Neurofibromatosis (von Recklinghausen's disease) is an autosomal dominant disease that affects one in 2,500-3,000 births. Because the head and neck is unique in its anatomical compartmentalization of the central and peripheral nervous systems, the potential for involvement of these areas with neurofibromas is substantial and accounts for a myriad of clinicopathologic presentations. In the present study, the incidence of involvement in the head and neck is 37%. We found 81% of the patients had café au lait spots, 63% had positive family histories, and 66% had multiple cutaneous nodules. An incidence of malignant transformation was documented in 3.5% of the cases. Management of benign neurofibromatosis must be individualized for each patient and ranges from incisional biopsy to wide local excision. In every case, the risk of severe neurologic deficit must be weighted against the benefits of surgical resection, which is usually incomplete. Genetic counseling is advised for all patients with this disease, since no cure is available.