A 52-year-old woman with schwannomatosis in the left sciatic nerve is presented. The patient had no stigmata of neurofibromatosis (NF) type 1 or 2. Cutaneous or spinal schwannomas were not detected. Magnetic resonance (MR) imaging of the sciatic nerve revealed more than 15 tumors along the course of the nerve. Histological examination revealed schwannomas consisting of Antoni A and B areas. Immunohistochemical study showed most cells reacting intensely for S-100 protein. The patient underwent conservative follow-up treatment due to the minimal symptoms. The relationship of the disease with NF-2 and plexiform schwannoma is discussed.