The article focuses on the epidermal nevus syndrome. The epidermal nevus syndrome (ENS) is characterized by the association of epidermal nevi with abnormalities in other organ systems. Most reported abnormalities have been in the central nervous system, skeletal system, eyes, and oral cavity. The most common epidermal nevi in ENS are verrucous nevi and sebaceous nevi. Skeletal and ocular abnormalities also are common in ENS. The germinitive cells give rise not only to keratinocytes, but also to the adnexal structures of the skin. Patients, especially, neonates, with an epidermal nevus should be evaluated carefully for other anomalies.