Abstract Of the various forms of neurofibromatosis, the best known are peripheral neurofibromatosis (von Recklinghausen's disease) and central neurofibromatosis. There is a rare form called segmental neurofibromatosis, in which the cutaneous and neural changes are limited to one sector of the body and with which there is usually no family history of neurofibromatosis. We report four patients with this disorder and stress the value of repetitive, limited excision of peripheral nerve tumors in their management.