The epidermal nevi are congenital skin lesions that consist of slightly raised ovoid or linear plagues. Smaller lesions occur most frequently on the face, scalp and neck, while larger lesions may affect any areas, There is often a predilection for the midline.

The epidermal nevus syndrome (ENS) is a rare neurocutaneous disorder that consists of epidermal nevi and a wide variety of congenital anomalies of the brain, eye, skeleton, and other systems.

Since 1964, about 60 cases of ENS have been reported in other countries and there was only one case report on ENS accompanied by multiple skeletal abnormalities and hydrosalpinx in Korea.

For the first time in Korea, We identified a patient with a neurologic syndrome consisting of epidermal nevi. left ptosis, left cryptorchidism, right ectopic testis, direct lateral abdominal wall hernias on both sides, right hemiparesis and mental retardation.

For this report, we describe data on the patient with ENS based on clinical description and pathologic studies.