Describe healthcare utilization (HCU) and costs at end-of-life among Medicaid beneficiaries with Huntington’s disease (HD). This retrospective cohort study used 2010‒2014 Medicaid Analytic eXtract files for 17 states. We identified non-dual-eligible Medicaid beneficiaries with HD, defined as having ≥1 medical claim with an HD diagnosis (ICD-9-CM: 333.4) between 2010 and 2014, and who died between 2011 and 2014. Date of death was defined as the index date. Beneficiaries had continuous enrollment in Medicaid fee-for-service for 1-year pre-index (baseline). Disease stage (early, middle, late) and comorbidities were measured at baseline. All-cause and HD-related HCU and costs (2014 USD) were assessed in the quarter preceding death (Q1) and just prior (Q2). We identified 1,247 Medicaid beneficiaries with HD who died between 2011 and 2014, of whom 128 were non-dual-eligible Medicaid beneficiaries that had continuous enrollment in the year prior to death. Mean (standard deviation [SD]) age at death was 51.2 (11.7) years and most beneficiaries were female (63.3%). At baseline, most beneficiaries were in the late stage of disease (93.8%), had a mean (SD) Charlson comorbidity index of 2.0 (2.4), and had 4.9 (2.3) chronic conditions. In the last two quarters (Q1, Q2) preceding death, hospitalizations (33.6%, 18.8%), emergency department visits (49.2%, 33.6%), skilled nursing facility visits (63.3%, 64.1%) and hospice care (57.0%, 26.6%) were common, as was use of antipsychotics (56.3%, 59.4%), antidepressants (45.3%, 46.1%), antiepileptics (47.7%, 49.2%) and anxiolytics (41.4%, 39.1%). Mean (SD) all-cause costs in this period were $27,552 ($15,649) and $25,041 ($16,171), respectively, which were driven by inpatient costs. HD-related costs contributed to approximately 70% of total costs in the months preceding death. This is the first study quantifying end-of-life burden among Medicaid beneficiaries with HD. Medicaid beneficiaries with HD have substantial HCU and costs in the final months of life, primarily resulting from inpatient care.