Epidermal nevus syndrome is a group of congenital neurocutaneous disorders characterized by epidermal nevi in association with cerebral, ocular, skeletal, and sometimes cardiac and renal abnormalities. These nevi have been classified according to their predominant component. We described a child presenting with inflammatory linear verrucous epidermal nevus on the head, which is an uncommon location. He also encountered ocular and central nervous system structural disorders.