Angiosarcoma of the scalp associated with Xeroderma pigmentosum

Xeroderma pigmentosum (XP) is a rare autosomal recessive genodermatosis associated with hypersensitivity to ultraviolet light due to defects in Deoxyribonucleic acid (DNA) repair. These patients have more than a 1000-fold increased risk of developing skin cancers. Although multiple cutaneous malignancies are common, the simultaneous occurrence of angiosarcoma and basal cell carcinoma is a rare phenomenon. We report a case of a 25-year-old male with XP with angiosarcoma scalp and basal cell carcinoma of face and occiput and discuss the treatment of this aggressive neoplasm with a review of the literature pertaining to it.