RESUMO
Segmental neurofibromatosis is a rare skin disorder characterized by neurofibromas and/or cafe-au-lait spots, limited to one dermatome of the body1. This disorder was first described by Gammel2 in 1931. The neurofibromas of segmental neurofibromatosis are commonly occupied by either a thoracic or a cervical dermatome and are unilateral, occuring more often on the right side than the left in young adults. According to Korean Dermatologic literature, 11 cases of segmental neurofibromatosis were reported. Among them, 7 cases showed unilateral involvement and 4 cases showed bilateral dermatomal involvement. We report a case of segmental neurofibromatosis which developed in a 76-year-old female. The skin lesions were situated on the left parietal scalp of the C2 dermatome, and had not been previously reported. We recommended surgical excision, but the patient refused further treatment.