Nevus sebaceous of Jadassohn (NSJ) is a complex hamartoma located on the face and scalp. Its incidence ranges between 0.05 and 1%. At birth, it is a well-circumscribed solitary yellowish, verrucous patch. At puberty, it becomes larger, more elevated, and cerebriform. Numerous secondary neo- plasms may occur in NSJ with an estimated rate from 10 to 30% increasing with age. The most common evolution is toward benign or malignant basaloid neoplasms. However, malignant basaloid degeneration is uncommon before puber- ty with only 10 documented cases of transformation into basal cell carcinoma (BCC).