Three patients with multiple neurilemmoma are presented. None of them have affected family with von Recklinghausen's disease. Two of them had multiple tumors in one peripheral nerve without café au lait spots. On the basis of this finding, we propose that the pathogenesis of these two cases is partial mutation of cell line of Schwann cell and that they are closely related to von Recklinghausen's desease. The other case is diagnosed as von Recklinghausen's desease (a central form) because of bilateral acoustic tumors.