Aims: To describe a case of xeroderma pigmentosum (XP) in a middle-aged African-American woman, and to review pertinent literature on this rare clinical scenario. Methods: Case report and English literature review related to XP in black patients. Results: A 34-year-old African-American woman diagnosed with XP with first cutaneous squamous cell carcinoma (SCC) at age 23 years progressed to develop additional SCC, basal cell carcinomas, and melanoma in situ. She was treated with sun avoidance and protection and required multiple excisions. Conclusions: We report a case of XP with a rare presentation in an African-American with onset of cutaneous neoplasm in her third decade. Severe photosensitivity and common malignancy seen in XP patients necessitate frequent surveillance with a low threshold for intervention. This case highlights the ability of XP to present in this demographic with a diverse spectrum of malignancies, and a potentially prolonged clinical course.
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