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Background and Aims Neuropathic pain is a largely underrecognized clinical feature of Huntington’s disease (HD), a rare inherited neurodegenerative disorder classically characterized by progressive motor, cognitive, and psychiatric impairments. Although current evidence strongly suggests that HD disrupts pain modulation pathways, no clinical case of neuropathic pain directly linked to HD had been published prior to our report. This study aims to report the first documented case of neuropathic pain in HD and to review the literature on the pathophysiological mechanisms supporting this association.Methods We present the case of a 74-year-old man with genetically confirmed, advanced-stage HD who exhibited persistent behavioral signs suggestive of pain. Due to severe dysarthria and cognitive decline, pain assessment relied on caregiver interviews and validated observational tools, including the ALGOPLUS scale. A focused clinical examination and diagnostic exclusion were performed. In parallel, a narrative literature review was conducted to compare our clinical findings with current physiopathological data.Results The patient exhibited consistent non-verbal signs of pain and mechanical allodynia in specific dermatomes. No alternative etiology was identified. Although the DN4 could not be administered, clinical signs strongly supported a diagnosis of neuropathic pain. These findings are consistent with reported degeneration of pain-related brain regions (prefrontal cortex, anterior cingulate cortex, striatum) and spinal cord abnormalities. Recent studies also implicate HAP1 dysfunction, whose inhibition in preclinical models increases mechanical pain sensitivity, as observed in our patient.Conclusions This case and literature review highlight neuropathic pain as a likely but underrecognized aspect of the HD phenotype. It underscores the importance of clinician awareness, the need for tailored pain assessment strategies in non-speaking patients, and the development of therapeutic approaches based on the specific pathophysiological mechanisms underlying pain in HD.