Abstract:A 33‐year‐old woman had multiple basal cell epitheliomas complicating a large, unilateral nevus sebaceus. Other unilateral epidermal nevi and a contralateral pigmentary nevoid disorder were noted on cutaneous examination. There were signs of skeletal, neurologic, and vascular involvement. This patient illustrates the unpredictable features of the epidermal nevus syndrome and the importance of close follow‐up to manage attendant complica