We report a case of epidermal nevus syndrome. The patient was a 26-year-old female who had numerous linear verrucous plaques on her neck, upper back and anterior chest. Biopsy of these lesions revealed epidermal hyperkeratosis, acanthosis and papillomatosis. Variable sized cafe-au-lait spots were scattered around the verrucous nevi. The other associated findings were claw hand deformity and epilepsy. Electroencephalogram showed very irregular, random and slow waves confined to the right temporooccipital area.