A Japanese man, who was 61 years of age, presented with multiple neurilemmoma in both legs which had arisen from different levels of both sciatic nerves. There were no clinical findings relating to von Recklinghausen's disease in the patient or his siblings. Multiple neurilemmoma may be the result of embryonic metameric anomalies. The condition may be distinguished from von Recklinghausen's disease by the distribution of the lesions and genetic analysis.