A 15-year-old female patient presented with extensive, bilateral systematized, verrucous plaques, involving the face, trunk, both extremities, and anogenital area. The onset of the lesions were at the age of 2 months. Abdominal CT showed the absence of the left kidney, teratoma of the right ovary and left ovarian cyst.

This is a case of epidermal nevus syndrome, which was composed of extensive epidermal nevi, congenital solitary kidney, left ovarian cyst and right ovarian teratoma.