Washington, DC 20549
Pursuant to Section 13 or 15(d) of
The Securities Exchange Act of 1934
Date of Report (Date of earliest event reported): March 23, 2023
Biohaven Ltd.
(Exact name of registrant as specified in its charter)
British Virgin Islands001-41477Not applicable
(State or other jurisdiction of incorporation)(Commission File Number)(IRS Employer Identification No.)
c/o Biohaven Pharmaceuticals, Inc.
215 Church Street
New Haven, Connecticut 06510
(Address of principal executive offices, including zip code)
(203) 404-0410
(Registrant’s telephone number, including area code)
Not applicable
(Former name or former address, if changed since last report)
Check the appropriate box below if the Form 8-K filing is intended to simultaneously satisfy the filing obligation of the registrant under any of the following provisions:
 Written communications pursuant to Rule 425 under the Securities Act (17 CFR 230.425)
 Soliciting material pursuant to Rule 14a-12 under the Exchange Act (17 CFR 240.14a-12)
 Pre-commencement communications pursuant to Rule 14d-2(b) under the Exchange Act (17 CFR 240.14d-2(b))
 Pre-commencement communications pursuant to Rule 13e-4(c) under the Exchange Act (17 CFR 240.13e-4(c))
Securities registered pursuant to Section 12(b) of the Act:
Title of each classTrading symbolName of each exchange on which registered
Common Shares, no par valueBHVNNew York Stock Exchange
Indicate by check mark whether the registrant is an emerging growth company as defined in Rule 405 of the Securities Act of 1933 (§230.405 of this chapter) or Rule 12b-2 of the Securities Exchange Act of 1934 (§240.12b-2 of this chapter).
Emerging growth company 
If an emerging growth company, indicate by check mark if the registrant has elected not to use the extended transition period for complying with any new or revised financial accounting standards provided pursuant to Section 13(a) of the Exchange Act.  

Item 7.01 Regulation FD Disclosure
On March 23, 2023, Biohaven Ltd. published an updated investor presentation (the “Presentation”) to its website. A copy of the Presentation is attached as Exhibit 99.1 to this Current Report on Form 8-K, and is incorporated herein by reference.

The information in this Current Report on Form 8-K, including the information set forth in Exhibit 99.1, is being furnished and shall not be deemed “filed” for purposes of Section 18 of the Securities Exchange Act of 1934, as amended (the “Exchange Act”), nor shall it be deemed incorporated by reference in any filing under the Securities Act of 1933, as amended, or the Exchange Act, except as shall be expressly set forth by specific reference in such a filing.

Item 9.01    Financial Statements and Exhibits.

(d) Exhibits

Exhibit Number*Exhibit Description
104The cover page of this Current Report on Form 8-K formatted as Inline XBRL.

*    The XBRL instance document does not appear in the interactive data file because its XBRL tags are embedded within the inline XBRL document.

Pursuant to the requirements of the Securities Exchange Act of 1934, the registrant has duly caused this report to be signed on its behalf by the undersigned hereunto duly authorized.
Date: March 23, 2023
Biohaven Ltd.
By:/s/ Matthew Buten
Matthew Buten
Chief Financial Officer

Investor Presentation March 2023

Forward-Looking Statement This presentation includes forward-looking statements within the meaning of the Private Securities Litigation Reform Act of 1995, including statements about Biohaven Ltd. (the “Company”) and our planned and ongoing trials for our BHV-2100, troriluzole, BHV- 5500, BHV-1100, BHV-1200, Taldefgrobep Alfa, BHV-8000, BHV-7000 and BHV-7010 development programs, the timing of and the availability of data from our clinical trials, the timing and our decisions to proceed with our planned regulatory filings, the timing of and our ability to obtain regulatory approvals for our product candidates, the clinical potential utility of our product candidates, alone and as compared to other existing potential treatment options, and the potential advancement of our early phase programs including ARM™, MATE™, MODE™, TRPM3, TDP-43, UC1MT, TYK2/JAK1, and Kv7. The use of certain words, including “continue”, “plan”, “will”, “believe”, “may”, “expect”, “anticipate” and similar expressions, is intended to identify forward-looking statements. Investors are cautioned that any forward-looking statements, including statements regarding the future development, timing and potential marketing approval and commercialization of our development candidates, are not guarantees of future performance or results and involve substantial risks and uncertainties. Actual results, developments and events may differ materially from those in the forward-looking statements as a result of various factors including: the expected timing, commencement and outcomes of Biohaven's planned and ongoing clinical trials; the timing of planned interactions and filings with the Food and Drug Administration; the timing and outcome of expected regulatory filings; complying with applicable U.S. regulatory requirements; the potential commercialization of Biohaven's product candidates; the potential for Biohaven's product candidates to be first in class or best in class therapies; and the effectiveness and safety of Biohaven's product candidates. You should, therefore, not rely on these forward-looking statements as representing our views as of any date subsequent to the date of this presentation. Additional important factors to be considered in connection with forward-looking statements are described in the Company’s filings with the Securities and Exchange Commission, including within the sections titled “Risk Factors” and “Management’s Discussion and Analysis of Financial Condition and Results of Operations”. This presentation also contains market data and other information based on industry publications, reports by market research firms or published independent sources. Some market data and information is also based on the Company’s good faith estimates, which are derived from management’s knowledge of its industry and such independent sources referred to above. Biohaven | Investor Presentation2 March 2023

Pfizer Acquisition of CGRP Platform $13B

Because we are passionate about the work we do everyday — FRANCINE Through state- of-the-art drug discovery and passionate scientists — LARRY With a powerful R&D engine — JAVIER We never forget patients need us. — JEREMY With a commitment to transforming patients’ lives — KATRINA

HIGH VALUE PLATFORMS INNOVATIVE PORTFOLIO PROVEN BUSINESS FORMULA Pursuing novel paths of science to transform the treatment of neurological and neuropsychiatric diseases A broad therapeutic portfolio addressing patient needs with intention. Formula for continued growth built upon past success of experienced team and a resilient focus on creating value for patients and shareholders

6 INDICATION PRECLINICAL PHASE 1 PHASE 2 PHASE 3 FILED ION CHANNEL: Kv7 ACTIVATOR Kv7 ION CHANNEL: TRPM3 INHIBITOR TRPM3 INFLAMMATORY: TYK2/JAK1 INHIBITOR TYK2/JAK1 GLUTAMATE PLATFORM Troriluzole MYOSTATIN PLATFORM BISPECIFIC TARGETED CELL THERAPY CD-38 DISCOVERY RESEARCH IgG Degrader IgA Degrader Next-Gen ADC Platform BHV-4157 | Spinocerebellar Ataxia (SCA) BHV-4157 | Obsessive-compulsive Disorder (OCD) BHV-2000 | Spinal Muscular Atrophy (SMA) BHV-1100 | Multiple Myeloma BHV-7000 | Epilepsy, Bipolar Disorder BHV-7010 | Epilepsy, Mood Disorders BHV-2100 | Chronic Pain Disorders BHV-8000 | Immune- Mediated Brain Disorders

Key Programs

Kv7 Ion Channels: Key Regulators of Neuronal Function 5 FAMILY SUBTYPES Primary localizations: • Kv7.1: cardiac • Kv7.2: CNS BHV-7000/7010 activator • Kv7.3: CNS BHV-7000/7010 activator • Kv7.4: smooth muscle and inner ear • Kv7.5: vascular tissue, neurons, skeletal muscle Kv7 Stria Vascularis Organ of Corti Vascular tissue Gastrointestinal tract Adventitia Media Intima Inner Ear Cardiac Muscle Skeletal Muscle Neuron Axon initial segment Node of Ranvier Synapse Serosa Muscle layer Mucosa Submucosa Biohaven | Investor Presentation8 Source: PHYSIOLOGY 26: 365–376, 2011; doi:10.1152/physiol.00009.2011 (Maria Virginia Soldovieri,1, Francesco Miceli,2,3 and Maurizio Taglialatela1,2) March 2023

Kv7 Potassium Channels Regulate Neuronal Excitability and Loss of Function Causes Epilepsy Biohaven | Investor Presentation9 Impaired Kv7 Channel Activity Causes Certain Types of Epilepsy Kv7 Activation Normalizes Action Potential Firing Loss-of-function mutations in Kv7.2 channel Abnormal EEG with Epileptic Activity Dalen Meurs-van der Schoor, Front Pediatr (2014). Hyperexcitability — Epilepsy and Mood Disorders Adapted from Wulff, Nat Rev Drug Discov (2009) . Increased frequency of action potential firing Normal frequency of action potential firing Kv7 channel activator Kv7 March 2023

Biohaven | Investor Presentation10 Kv7 PLATFORM Focal Epilepsy Potential Best-in-Class, Fast-Follower Approach Clinically validated mechanism of action Pursuing Differentiation Preclinical data suggests wide therapeutic index in the clinic by dialing out GABA risk (somnolence, sedation, dizziness) Translatability Preclinical data in epilepsy models is predictive of clinical efficacy Broad Potential in Adjacent Indications Warrants further evaluation in BPD, depression, pain, others Patent Protection Both BHV-7000 and BHV-7010 covered until 2039 Status Update BHV-7000 Phase 1 SAD/MAD study completed (Canada) BHV-7010 IND anticipated in 2023 March 2023

BHV-7000 is chemically stable to photo-oxidation BHV-7000 is selective for Kv7 over GABAA receptors a BHV-7000 has a wide therapeutic index preclinically a 1st and 2nd Gen Kv7 Activators Show Clinical Anti-seizure POC, But Off-target Activities, Opportunity for 3rd Gen Kv7 Differentiation XEN1101 • XEN1101 and ezogabine significantly greater GABAA receptor allosteric activators than BHV-7000 in vitroa • GABAA receptor activation: somnolence, dizziness, fatigue, diplopia Ezogabine XEN1101 BHV-7000 G A B A A A ct iv at io n a (% B as el in e) 0 20 40 60 GABAA Posi ive Allosteric Modulation % In cr ea se fr om b as el in e 10 µM BHV-7000 10 µM EZO 10 µM XEN1101 ✱✱ ✱✱ 0 20 40 60 GABAA Positive Allosteric Modulation % In cr ea se fr om b as el in e 10 µM BHV-7000 10 µM EZO 10 µM XEN1101 ✱✱ ✱✱ ** ** ** significantly different from BHV-7000, all tested at 10 µM EZOGABINE • Unstable when exposed to light • Label warnings for skin discoloration • Black box warning for retinal abnormalities/vision loss FDA Drug Safety Communication: Potiga (ezogabine) [04-26-2013] BHV-7000 • Potent activator of Kv7 channels • Effective and well-tolerated in preclinical seizure assays Th er ap eu tic In de x Ezogabineb XEN1101b BHV-7000a 10 20 30 40 50 Kv7 Biohaven | Investor Presentation11 a. Biohaven data on file (2022). b. Calculated as ratio of TD50 (rotarod) to ED50 (MES seizure assay) data presented by Xenon at Epilepsy Foundation Pipeline Conference, San Francisco (Feb 2018). Preclinical results are consolidated from separate reports and graphed together. Data presented is not the result of any head-to-head clinical trials and does not mean or suggest that BHV-7000 is clinically more safe and effective. March 2023

BHV-7000 Exhibits Highly Differentiated Preclinical Profile Biohaven | Investor Presentation12 Wide Therapeutic Index Kv7.2/7.3 Activator GABAA Activity “dialed-out” Ezogabine XEN1101 BHV-7000 Activator, clinical and preclinical anti-seizure activity Activator, clinical and preclinical anti-seizure activity Activator, clinical and preclinical anti-seizure activity GABAA activity present GABAA activity present Negligible GABAA activity <3x reporteda,b >40xb<5x reporteda Kv7 a. Calculated as ratio of TD50 (rotarod) to ED50 (MES seizure assay) data presented by Xenon at Epilepsy Foundation Pipeline Conference, San Francisco (Feb 2018). b. Biohaven data on file (2022). Preclinical results are consolidated from separate reports and not a result from head-to-head comparisons Data presented is not the result of any head-to-head clinical trials and does not mean or suggest that BHV-7000 is clinically more safe and effective. March 2023

BHV-7000 Phase I SAD/MAD Study Update: Dosing Completed Preliminary Safety • Single doses up to 100 mg and multiple doses up to 40 mg daily x15 days generally well-tolerated • Most AEs mild and resolved spontaneously • No serious or severe AEs • No dose limiting toxicities Preliminary Pharmacokinetics • Target concentrations for efficacy exceeded based on preclinical MES model • High fat meal had no effect on exposures Pooled adverse events from SAD and MAD cohorts occurring in more than 1 subject (preliminary blinded data including BHV-7000 and placebo groups) Biohaven | Investor Presentation13 Kv7 Pooled Adverse Events* N = 61 Headache 7 (11.5) Back pain 3 (4.9) Constipation 2 (3.3) Abdominal discomfort 2 (3.3) Urinary frequency 2 (3.3) * MedDRA® Preferred Term March 2023

BHV-7000 Shows Favorable CNS-Associated AE Profile Compared to Xen1101 Across Pooled MAD Cohorts 14 1MedDRA® Preferred Term within the System Organ Class of “Nervous System Disorders” 2Data from 2019 73rd Annual American Epilepsy Society Meeting poster, Abstract # 3.31, published 11.25.19 Data presented is not the result of any head-to-head clinical trials and does not mean or suggest that BHV-7000 is clinically more safe and effective. Pooled Adverse Events1 BHV7000-101 MAD pooled (active, n=17) Xen1101 MAD pooled2 (active, n=18) Somnolence 0% 39% Headache 18% 39% Balance disorder 0% 17% Dizziness 0% 17% Memory impairment 0% 28% Sensory disturbance 0% 11% Speech disorder 0% 33% Biohaven | Investor Presentation March 2023

Maximal Electroshock Seizure Model: Strongly Positive Predictive Translation to the Clinic Predictive Efficacy: Efficacy results from the maximal electroshock (MES) model are predictive of efficacy in the epilepsy patient population. Other CNS diseases have not benefitted from such similarly predictive animal models (Loscher, 2016). Kv7 Biohaven | Investor Presentation15 March 2023

Kv7 In mania models, Kv7 activation reduces hypermetabolism and hyperactive behavior1 Robust MDD efficacy likely to translate to bipolar disorder2 fMRI study: ezogabine effects mediated by the limbic system Preclinical and Clinical Data Suggest a Role for Kv7 in Bipolar Disorder and Depression Biohaven | Investor Presentation16 1. Kristensen et al. J Neurochem (2012). 2. Costi et al. Am J Psychiatry (2021); 178(5):437-446. 3. Golberg et al. J Clin Psychiatry (2021) 82(1):20ed13649 DECREASING BRAIN HYPERACTIVITY Retigabine normalizes a mania model hypermetabolic signature to a higher degree than lithium Mania 3/3 positive studies of fluoxetine in bipolar depression indicate effects in MDD translate to bipolar disorder3 Depression Vehicle Lithium 120 mg/kg Retigabine 10 mg/kg ~7pt decrease vs placebo with ezogabine in depression symptoms Ch an ge in M AD RS sc or e Weeks 0 -5 -10 -15 -20 0 1 2 3 4 5 Ezo gab ine Plac eb o 0.8 0.6 0.4 0.2 0.0 -0.2 -0.4 Ch an ge in V S ac tiv at io n to re w ar d an tic ip at io n (o ut co m e > ba se lin e) March 2023

Epilepsy Blockbuster Peak Sales Potential for Kv7 Platform Mood Disorders Biohaven | Investor Presentation17 ✓ Easy to Prescribe Convenient dosing, no titration, no food effect ✓ Safe/Tolerable Side effects negatively impacted other recent epilepsy launches ✓ Biohaven Proven Commercial Expertise in Neuroscience 1. (1.2% of the population has epilepsy, 60% is focal onset). 2. Kammerman S. West J Med. 2001 Aug;175(2):99-103. 3. Hauser WA. Epilepsia. 1991 Jul-Aug;32(4):429-45. 4. (2.8% of US adults have had BPD within last year and 4.4% of population over their lifetime). 5. Hasin DS. JAMA Psychiatry. 2018 Apr 1;75(4):336-346. Kv7 34.5M MAJOR DEPRESSIVE DISORDER (US)5 200–300 KCNQ2-DEE incidence in live births 3.5M EPILEPSY PATIENTS adult and pediatric, US1–3 1.1M 2.4M Treatment refractory/ uncontrolled patients Focal epilepsy patients 7–11M BIPOLAR DISORDER (US)4 March 2023

TRPM3 (BHV-2100): A Novel Peripheral Target for Neuropathic Pain Biohaven | Investor Presentation18 Differentiated from existing programs targeting TRPV1 and TRPA1 • Current SoC comes with liabilities — addiction (opioids), sedation • Pre-clinical data shows reversal of pain in various models, without sedative effects • IND planned for 2H 2023 Appeared effective, but class terminated for hyperthermia despite significant investment X Difficult target to drug with PK/solubility issues; remains in active drug development (Lilly) Conflicting data for pro- and anti-nociceptive role; remains in active drug development Promising as a novel target: expressed at similar levels as TRPV1 and TRPA1 in peripheral neurons but unlikely to have thermal liabilities See Koivisto et al, Nature Reviews Drug Discovery 2022 for background on TRP channel drug development. Peripheral sensory neuron Pain sensation CGRP March 2023

TRPM3 (BHV-2100) Reduces Pain in Preclinical Models of Chemotherapy and Diabetic Neuropathy Encouraging evidence of pain reduction without the sedation observed with high dose tramadol/gabapentin Biohaven | Investor Presentation19 BHV-2100 5 mg/kg BHV-2100 25 mg/kg Tramadol 5 mg/kg BHV-2100 1 mg/kg Vehicle BHV-2100 1 mg/kg BHV-2100 25 mg/kg Pregabalin 30 mg/kg Gabapentin 150 mg/kg BHV-2100 5 mg/kg Vehicle DISCOVERY RESEARCH Drug administered 7 days after diabetic induction with STZ in rats Drug administered 6 days after oxaliplatin in mice Source: Biohaven data on file March 2023

BHV-8000: TYK2/JAK1 in NeuroInflammatory Disease BHV-80001 has potential for first- in-class and best-in-class dual TYK2/JAK1 inhibition ü Most advanced, selective TYK2/JAK1 inhibitor with good brain penetration in animal models, to the best of our knowledge ü Dual inhibition of TYK2 and JAK1 can effectively block Th17 cell generation, Type I IFN signaling and inflammation ü JAK inhibitors are more efficacious anti-inflammatory agents than anti- TNFs Tyk2 Jak1 Jak2 → IL-17 Jak3 Modified from Gonciarz et al 2021 Immunotherapy (2021) 13(13), 1135–1150 March 2023Biohaven | Investor Presentation20 1. Formerly TLL-041

Cellular Drivers In Neuroinflammation: Predominant TYK2/JAK1 Effects TYK2/JAK1 cytokines • IFN-g • IL-b • TNF downstream of IFN-g • IL-8 • GM-CSF, MCP-1 Microglia are the resident macrophages of the CNS, playing an important role in neuroinflammation, repair and maintenance Microglia • IFN-g • IL-12 • TNF • IL-8 Abnormal astrocytic activity may exacerbate inflammatory reactions and contribute to tissue damage Astrocytes Lymphocytes, other leukocytes • IL-23 • IL-17 downstream of IL-23 • IL-2, IL-4 Strong evidence for Th17 lymphocyte involvement in the pathogenesis of multiple sclerosis and Parkinson’s disease March 2023Biohaven | Investor Presentation21

Properties of TYK2/JAK1 Selective Inhibitors Ideal for Treatment of Neuroinflammation Brain penetrance • Biologics are not brain penetrant • Requires specially designed small molecule to reach site of action • Existing agents exclude all lymphocytes from the CNS • TYK2/JAK1 inhibitor expected to selectively deplete CD4+ Th17 cells at the site of action Dose-limiting tolerability issues of JAK2 and JAK3 inhibition minimized • Serious cardiovascular events • Severe immunosuppression • Anemia, cancer, death Small molecules allowing convenient at-home, daily oral administration Potential adverse events of central immunomodulation rapidly addressed following withdrawal vs. long exposure of biologics March 2023Biohaven | Investor Presentation22

BHV-8000: Phase-1 Ready, Brain-Penetrant TYK2/JAK1 Inhibitor for Neurological Disorders BHV-8000 ü The most advanced brain-penetrant, selective, dual TYK2/JAK1 inhibitor • Good brain penetration in pre-clinical species • Excellent selectivity over JAK2 and JAK3, avoiding associated toxicities • Wide estimated therapeutic windows ü Mechanistic proof-of-concept has been demonstrated in preclinical models: • Inhibited LPS-induced proinflammatory cytokine production in mouse microglia • Efficacy in Th17-dependent EAE mouse models • Completely blocks dsRNA induced pSTAT1 signaling, considered important in Parkinson’s disease, AD and ALS ü Demonstrated good safety pharmacology and toxicology • Completed IND enabling toxicology studies ü Pharmacokinetics potentially suitable for once daily dosing March 2023Biohaven | Investor Presentation23

TYK2/JAK1 Pathways in Parkinson’s Disease • a-synuclein aggregates in dopaminergic neurons of substantia nigra • Microglia present a-syn fragments with MHC-II leading to immune response, and activated T cells entering the CNS • T cells release IFN-g, activating microglia which release further proinflammatory cytokines • Activated T cells and microglia injure and kill DA neurons • Strong evidence implicates IL-17 and Th17 cells in Parkinson’s • TYK2/JAK1 kinase inhibitors reduce Th17 cells (through IL-23 inhibition) and reduced microglial activation through inhibition of IFN-g signaling Reish and Standaert, a-syn induction of inflammation in PD; Fu et al. Journal of Neuroinflammation (2022) 19:98 BHV-8000 is ideally suited to reducing both important axes of neuroinflammation in Parkinson’s March 2023Biohaven | Investor Presentation24

TYK2/JAK1 Inflammatory Pathways in Multiple Sclerosis • Multiple sclerosis is an inflammatory disease in which humoral immune and cell-mediated immune responses target CNS antigens • IL-17A-defective mice are highly resistant to induction of EAE • PKM2 activators mediate potent inhibitory effects in EAE model due to Th17 cell effects • In a meta-analyses of literature, TNF-a, IL-15, IL-12, IL-23/IL-17, and IFNg were elevated in or predictors of MS patients vs. controls • Secukinemab (IL-17A) demonstrates an effect in RRMS • Brain penetrant TYK2/JAK1 kinase inhibitors reduce Th17 cells (IL-17 and IL-23) and target IL-12 signal transduction X X X X McGinley et al, Immunity 52:342-356, Palle et al, Med Sci, 5:23, 2017; Bai et al, Frontiers in Neuroscience, 10.3389, Oct 4, 2019, Havrdova, Multiple Sclerosis Journal, 18_509, 2012 Figure modified from BHV-8000 is ideally suited to reducing neuroinflammation in MS March 2023Biohaven | Investor Presentation25

TYK2/JAK1 Inflammatory Pathways in Other Neuroinflammatory Disorders • Alzheimer disease • Neuroinflammation is a key event in AD pathogenesis • Cytokines and chemokines affect TAU phosphorylation and amyloid deposition • Ab induces the glial cell expression of several pro- inflammatory cytokines, such as interleukin-1b (IL-1b), IL-6, tumor necrosis factor-a (TNF-a) and interferon-g (IFN-g) • Amyotrophic lateral sclerosis • TDP-43 inclusions may trigger Type I interferon in Amyotrophic Lateral Sclerosis and FTD • Autoimmune encephalomyelitis • Multiple cytokines overlapping with TYK2/JAK1 cytokines suggests likely therapeutic role Domingues et al, Curr Alzheimer Res. 2017 Aug; 14(8): 870–882. Jonas J. Neher, Immunity 55, May 10, 2022 BHV-8000 reduces several key cytokines driving Alzheimer’s pathology March 2023Biohaven | Investor Presentation26

BHV-8000 Summary CNS immunomodulation in neuroinflammatory disorders will interrupt the cycle of inflammation, neuronal injury and demyelination, and will complement other approaches directly addressing neurodegeneration such as amyloid, a-synuclein, tau and mitochondrial targeting therapies Strong evidence supports potential efficacy in Parkinson’s disease, multiple sclerosis, Alzheimer disease and possibly further neuroinflammatory diseases Selectivity for TYK2/JAK1 mitigates non-selective JAK class liabilities, largely related to JAK2 and JAK3 inhibition, and offer potential to improve benefit-risk for the highly selective BHV-8000 dual kinase inhibitor Selective inhibition of TYK2/JAK1 has profile for best-in-class immunomodulation in neuroinflammatory disorders March 2023Biohaven | Investor Presentation27

Biohaven | Investor Presentation28 TRORILUZOLE SCA and OCD SPINOCEREBELLAR ATAXIA (SCA) • Reduction in fall risk + treatment effect in SCA3 genotype • Safe and well-tolerated profile • Regulatory engagement planned for H1 2023 OBSESSIVE COMPULSIVE DISORDER (OCD) • Compelling mechanistic rationale for cortico-striatal glutamate abnormalities in OCD patients • Strong PoC in Phase 2 • Well-characterized in 1,000+ patients • Two Phase 3 studies ongoing; enrollment completion anticipated YE 2023 March 2023

Troriluzole Phase 3 Results: Treatment Effect in SCA3 Genotype • Post-hoc analysis suggests troriluzole has disease-stabilizing effect in SCA3 genotype (most common genotype, pre- identified randomization stratum) • In SCA3 patients (N=89 / 41%), numerical treatment benefit observed on change from baseline on f-SARA at week 48 • LS mean change difference -0.55, nominal p-value = 0.053, 95% CI: -1.12, 0.01 • In subgroup of SCA3 patients able to walk w/o assistance at baseline (N=55), greater numerical treatment benefit observed on change from baseline on f-SARA at week 48 (Figure) • LS mean change difference -0.71, nominal p-value = 0.031, 95% CI: -1.36, -0.07) • In SCA3 patients, reduction in fall risk also observed compared to placebo • Primary endpoint was not met in overall population (n=213) f-SARA: Functional Scale for the Assessment and Rating of Ataxia GLUTAMATE Reduction in fall risk + treatment effect in SCA3 patients suggest troriluzole has clinically meaningful benefit Biohaven | Investor Presentation29 March 2023

BHV-4157 Troriluzole Treated OCD Patients: Strong Signal Observed in Phase 2 POC Supports Advancement to Phase 3 STUDY BHV-4157-202 Patients with moderate-to-severe OCD (Y-BOCS score ≥ 19) and inadequate response to standard of care SAMPLE SIZE 226 subjects RANDOMIZATION 1:1 DOSE Troriluzole 200 mg QD vs Placebo QD (in patients on standard of care) PRIMARY OUTCOME Y-BOCS, precedented outcome measure accepted by FDA Table 1: Troriluzole Effect on OCD in Phase 2/3 Trial1 Y-BOCS Total Change from Baseline Week 4 (N=115a, 111b) 8 (N=108a, 96b) 12 (N=102a, 99b) a. Placeboa -2.9 -3.6 -4.9 b. Troriluzoleb -3.4 -5.1* -5.9 p-value 0.451 0.041 0.220 1. BHV-4157-202 Final Unblinded Analysis YBOCS Total Change from Baseline by Week LSMeans from MMRM Model MITT Data Set Table 2: Troriluzole Effect on Patients with Severe OCD1 Y-BOCS Total Change from Baseline Week 4 (N=47c, 49d) 8 (N=45c, 42d) 12 (N=43c, 44d) a. Placeboc -3.5 -3.1 -4.6 b. Troriluzoled -4.1 -6.0* -7.0 p-value 0.584 0.035 0.084 1. Patients at baseline with median Y-BOCS total scores > 26 (severe OCD symptoms). * p < 0.05 versus placebo Y-BOCS, Yale-Brown Obsessive Compulsive Scale (FDA accepted outcome measure) GLUTAMATE Biohaven | Investor Presentation30 March 2023

Biohaven | Investor Presentation31 TALDEFGROBEP ALFA SMA Pivotal Phase 3 Study Initiated July 2022 • Large pre-clinical and clinical safety package licensed from BMS • Limited additional work needed to support BLA submission • 34 sites total, ~20 open POC for Mechanism of Action Supported by clinical data and multiple disease models Muscle and Bone Improvements Observed in non-clinical studies Strong Supporting Efficacy Signal Muscle mass growth in healthy subjects Large Safety Database Established in prior pediatric studies March 2023

T-Alfa: Targeting a Differentiated Regulatory Pathway of Muscular Growth The Most Advanced Phase 3 Ready Myostatin Inhibitor Safety studied with more than 300 subjects dosed in prior clinical studies in pediatric and adult populations Non-clinical studies showed muscle and bone improvements POC for mechanism of action supported by clinical data and multiple disease models Phase 3 study initiated in July 2022 Myostatin regulates muscle growth negatively Taldefgrobep alfa binds to myostatin to inhibit signaling Mechanistically, taldefgrobep-myostatin complex acts as ActRIIB receptor antagonist MYOSTATIN Biohaven | Investor Presentation32 March 2023

• Harnesses the body’s own machinery for degrading proteins • Extracellular protein targets are eliminated via the asialoglycoprotein receptor (ASGPR) • Protein targets are degraded via endolysosomal proteolysis * formerly BH 2640 Bispecific Platform: IgG Degradation via MoDE™ March 2023Biohaven | Investor Presentation33 Molecular Degraders of Extracellular Proteins (MoDE): small molecules bind extracellular target proteins and cause them to be removed from the body through the liver Binds liver (ASGPR) Binds protein target Pathogenic target protein and MoDEs in circulation

Hepatic ASGPR Receptor Harnessed for Efficient and Safe Removal of Pathogenic Targets – Pan IgG Degradation March 2023Biohaven | Investor Presentation34 02 03 BHV-1300 administered by subcutaneous or IV BHV-1300 binds circulating IgG to ASGPR on hepatocyte* Internalized IgG is rapidly degraded in lysosomes Hepatocyte ASGPR cell membrane receptors rapidly renewed BHV-1300 01 Immunoglobulin G (IgG) HepatocyteBHV-1300 Asialoglycoprotein receptor (ASGPR) *Stylistic representation of IgG binding site Dial-able levels of IgG preserved

Hepatic ASGPR Receptor Harnessed for Efficient and Safe Removal of Pathogenic Targets • High capacity ASGPR hepatocellular receptors internalize plasma proteins with specific motifs • Bispecific ASGPR-binders with target-binder effectively removes [pathogenic] target from the circulation • IgG may be more rapidly removed from the circulation than FcRN inhibitory antibody or antibody fragments, without causing hypoalbuminemia or dyslipidemia • Improved, dialable potency (deeper IgG/IgA reductions possible) • Improved pharmacodynamics (faster onset of action) • Improved safety profile (fewer side-effects, rapid drug elimination) March 2023Biohaven | Investor Presentation35 BHV-1300: A highly optimized Biohaven ASGPR binder advancing as drug candidate ü Balances liver removal of unbound to target-bound drug ü Optimizes safety vs efficacy ü Improves kinetics of target removal ü Suitable Target Product Profile for a rapid onset medication with weekly or less frequent SC administration

HepG2 cells internalize IgG-BHV-1300 complexes In vitro BHV-1300 Mediates Removal, Uptake and Degradation of IgG March 2023Biohaven | Investor Presentation36 1 10 100 1000 0 20000 40000 60000 80000 nM M FI Uptake of IgG in HepG2 EC50 110 BHV-1300 125nM BHV-1300 Untreated Uptake of IgG in HepG2 1 10 100 1000 10000 0 20000 40000 60000 80000 nM M FI Uptake of IgG in HepG2 EC50 99 BHV-1300 1 10 100 1000 0 20000 40000 60000 80000 nM M FI Uptake of IgG in HepG2 EC50 110 BHV-1300

BHV-1300 human IgG depletion in mice, single dose 12 mg/kg BHV-1300 Selected for Remarkable Efficiency in Removal of Exogenously Administered Human IgG in Mouse Screen Approximately 40% IgG removal achieved in 2 hours with a molar ratio of drug-to-target = 1.0 March 2023Biohaven | Investor Presentation37 0 2 4 6 8 10 0 1×109 2×109 3×109 4×109 5×109 Hours Ig G p g/ m l BHV-1300 human IgG depletion in mice, single dose 12 mg/kg BHV1300 SD, 12 mg/kg +IgG IgG

IgG is Specifically Depleted: IgA and IgM Levels Remain Unchanged March 2023Biohaven | Investor Presentation38 0 100 200 300 400 0 100 200 300 Hours Ig M % T 0t IgM % change by group 0 mg/kg 10 mg/kg 30 mg/kg 75 mg/kg 150 mg/kg 250 mg/kg • Preliminary BHVN data and literature consistent with an absence of effects on serum albumin, LDL, HDL or triglycerides, and specificity for targeted IgG species • Remarkable drug efficiency in mouse given exogenous IgG recapitulated in monkeys with endogenous IgG. Molar ratio of approximately 1.0 allows 60% IgG lowering following a single dose BHV-1300 administered IV to cynomolgus macaques 0 5 10 15 0 50 100 150 200 Days % Ig G T0 IgG 0 5 10 15 0 50 100 150 200 Days Ig A % T 0t IgA 0 5 10 15 0 50 100 150 200 Days Ig M % T 0t IgMIgG % change by group IgA % change by group IgM % change by group Days

BHV-1300 Administered by SC, IM or IV Routes has Comparable Bioavailability March 2023Biohaven | Investor Presentation39 Potentially allows at- home, self administration with subcutaneous injection 0 10 20 30 10 100 1000 10000 100000 Hours ng /m l BHV-1300 levels in plasma after a SD of 10 mg/kg in the presence of human IgG BHV-1300 IV 10 mg/kg BHV-1300 SQ 10 mg/kg BHV1300 IM 10 mg/kg F SQ ~ 99% F IM ~ 93% BHV-1300 levels in plasma after a single dose of 10 mg/kg in the presence of human IgG

Pan-IgG Degrader (BHV-1300) and SoC (Efgartigimod) Primate Data Efgartigimod: Cynomolgus Pharmacodynamics 0 5 10 15 0 25 50 75 100 125 Days % Ig G , ba se lin e vehicle 10 mg/kg 75 mg/kg 250 mg/kg BHV-1300: Cynomolgus Pharmacodynamics 3 days 75% depletion 5–7 days 50% depletion March 2023Biohaven | Investor Presentation40 IND ready in H2 2023 The Journal of Clinical Investigation 2018:128(10):4372-4386. httos://

DISCOVERY RESEARCH Bispecific Platform: IgA Degrader for IgA Nephropathy Therapeutic Hypothesis: Galactosyl deficient-IgA (Gd-IgA) containing-immune complexes drive glomerular injury in IgA nephropathy.1 Specific removal of this pathogenic Gd-IgA with preservation of “normal” IgA (and other Ig’s) permits disease remission without incurring an infection risk. • Highly selective, rat mAb conjugate binds Gd-IgA with high affinity • Antibody humanization ongoing, subsequent conjugation with established degrader technology will allow rapid and efficient hepatic extraction, leaving “normal” IgA Biohaven | Investor Presentation41 1. Canetta et al, 2014, others. 2. Untested therapeutic hypothesis. anti-Gd-IgA antibody Liver Antibody-based MoDE removes Gd-IgA Gd-IgA removal drives efficacy without infection risk2 Chimeric antibody-ASGPR ligand conjugate specifically mediates endocytosis of Gd-IgA in HepG2 cells 0.1 1 10 100 1000 10000 0 200000 400000 600000 800000 1000000 nM M FI Chimeric KM55 ASGPR ligand conjugate specifically mediates endocytosis of galactose deficient IgA galactose deficient IgA normal serum IgA M ea n Fl uo re sc en ce In te ns ity March 2023

BHVN Bispecific Degrader Platform Summary • Pan-IgG degrader (BHV-1300) in nonclinical development with anticipated 2023 IND • Mechanism with potential for “pipeline in a product” • Highly optimized chemistry, drug delivery potential, and differentiated mechanism of action compared to standard of care • Following pan-IgG degraders, versatile and novel BHVN bispecifics for precision removal of degalactosylated IgA (pathogenic species of IgA in IgA nephropathy) and removal of other pathogenic proteins including specific autoantibodies currently in late discovery March 2023Biohaven | Investor Presentation42

Bispecific Platform: CD38 Targeted Cell Therapy for Multiple Myeloma CD38 Antibody Recruiting Molecule (ARM™) • Modular bispecific molecules with two moieties, each designed for non-covalent binding to a specific target • Redirect endogenous antibodies to target cancerous or virally infected cells for immune destruction Biohaven | Investor Presentation43 Key Potential Advantage Over Biologics ü Lower manufacturing cost ü More versatile — smaller and tunable ü Faster and less expensive to develop ü Better safety and efficacy • Non-immunogenic; better dosing • Enhanced PK properties • Reduced NK cell fratricide compared to daratumumab ARM BHV-1100 CD38 Targeting Therapy Mult iple Mye lom a Cell Multipl e Myelo ma Cell Multiple Myeloma CellNK Cell BHV-1100 + CIML NK cell CD38 ARM™ Binds to CD38 receptor on the surface of multiple myeloma cells March 2023

Ex Vivo Patient Study: 4 Out of 5 Patients Showed Reduction in Plasma Cells (Multiple Myeloma) Biohaven | Investor Presentation44 ARM Using patient bone marrow and a combination therapy % reduction in plasma cells in patient bone marrow +/- autologous CIML NK cells in presence of BHV-1100 0 20 40 Patient 1 Patient 2 Patient 3 Patient 4 Patient 5 % re du ct io n in C D 38 + C D 13 8+ P C * * * * Patient blood samplePatient bone marrow Isolate NK cells 1. Bone marrow 2. BHV-1100 4 hrs 1. Bone marrow 2. BHV-1100 3. CIML NK cells Readout % CD38+ CD138+ plasma cells Generate CIML NK cells overnight ONGOING CLINICAL STUDY MRD + post-transplant multiple myeloma patients ongoing at Dana Farber Institute • First patient survival to one year • Two additional patients randomized Journal of Clinical Oncology 2020 38:15_suppl, 8523-8523. BHV-1100 + no NK cells BHV-1100 + CIML NK cells March 2023

Second-generation ADC-linker technologiesBiohaven third-generation technology DISCOVERY RESEARCH Bispecific Platform: Advancing Next-Generation, Site-Specific Antibody Drug Conjugates (ADCs) Potential for best-in-class A single residue per heavy chain is available for conjugation • Controlled DAR* ratio is critical to therapeutic index • MATE tech precisely defines DAR* Improved linker stability should yield wider therapeutic index • Improved safety: less systemic, untargeted payload • Improved efficacy: targeted payload delivered to tumor Uses native antibody: potentially improved CMC vs. current tech N N N N O O O Payloadn n O O O Payload N H Click chemistry to engineered antibody Biohaven | Investor Presentation45 *DAR = Drug antibody ratio 0 0.1 0.2 0.3 0.4 0.5 1 2 3 4 5 6 Fr ac tio n La be lle d Residue Peptide Mapping (typical cartoon) 0 0.2 0.4 0.6 0.8 1 1 2 3 4 5 6 Fr ac tio n La be lle d Residue Peptide Mapping (typical cartoon) March 2023

Capitalization Considerations CGRP royalties: Pfizer will make royalty payments in low- to mid-teens% in respect of annual U.S. net sales of rimegepant and zavegepant >$5.25B, subject to annual cap ($400M/year)3 ~$465.3 million2 SHARE O/S CASH & MARKETABLE SECURITIES POTENTIAL ROYALTIES 1. Excludes 9.1 million options ($7.00 strike price). 2. As of December 31, 2022. 3. Cap reached if aggregate annual U.S. net sales of rimegepant and zavegepant amount to $8.15B. Royalty payments would be in respect of years ended on or before 12/31/40. Biohaven | Investor Presentation46 68.2 million1 March 2023

File IND Near-Term Milestones (Anticipated) 47 DRUG NAME INDICATIONS 2H 2022 1H 2023 2H 2023 BHV-7000 Kv7 channel activator Focal Epilepsy Bipolar Disorder BHV-7010 Kv7 channel activator Epilepsy and Mood Disorders BHV-2100 TRPM3 Chronic Pain BHV-8000 TYK2/JAK1 Immune-Mediated Brain Disorders Troriluzole NCE prodrug of riluzole Spinocerebellar Ataxia Obsessive-Compulsive Disorder Taldefgrobep Alfa Anti-myostatin adnectin Spinal Muscular Atrophy BHV-1300 IgG degrader Undisclosed Milestone achieved Regulatory Interaction Complete Enrollment Initiated Phase 3 Initiate Phase 2/3Initiate EEG StudyPhase 1 Topline Initiate Phase 2/3 File IND File IND Initiate Phase 1 March 2023Biohaven | Investor Presentation

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