BACKGROUND: Xeroderma pigmentosum (XP) is a rare autosomal recessive DNA-repair disorder characterized by extreme ultraviolet radiation (UVR) sensitivity, markedly increased cutaneous malignancy risk, and progressive neurological disease in approximately one-third of patients. Emerging evidence suggests an increased risk of internal malignancies, particularly primary central nervous system (CNS) tumors. OBJECTIVE: The aim of this work was to describe primary CNS tumors in patients with XP managed at the UK National XP Service and to review the literature. METHODS: We retrospectively identified five genetically confirmed XP patients with primary CNS tumors followed longitudinally within the multidisciplinary UK National XP Service, including regular neurological evaluation. RESULTS: Five patients (aged 20-79 years) developed primary CNS tumors: three XP-C and two XP-A. Tumors included gliomas, meningiomas, and subependymoma. CNS tumors occurred predominantly in younger patients with XP-C, whereas late-onset, slow-growing meningiomas were observed in milder XP-A phenotypes. One patient with XP-C died of glioblastoma. CONCLUSIONS: XP is associated with primary CNS tumor risk, particularly in XP-C and late-onset XP-A, supporting regular neurological assessment and targeted neuroimaging. © 2026 The Author(s). Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.