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Abstract:  Nevus sebaceous usually occurs as a solitary lesion located mostly on the scalp and the face. The potential for nevus sebaceous to develop malignancy is well recognized, though it is generally thought that this transformation occurs only postpubertally or later. However, few cases of basal cell carcinoma arising in nevus sebaceous before puberty were reported. Herein, we report a case of basal cell carcinoma arising in a nevus sebaceous on the scalp of a 10-year-old boy, and a review of the literature on prepubertal cases and the need for prophylactic surgery.