ABSTRACT
We are presenting a familial schwannomatosis without the features of neurofibromatosis (NF). We retrospectively reviewed the hospital charts, radiology films, operative notes and pathology slides of the patient. There was a family history of schwannomatosis. The patient had contrast-enhanced MRI, which was negative for vestibular schwannomas. The patient underwent surgical excision of symptomatic lesions. Histopathology confirmed these lesions as schwannomas consisting of areas of Antoni A and B, and immunohistochemical study was positive for S-100 protein. We recommend surgery for symptomatic lesions. Asymptomatic tumours can be monitored. Regular follow-up is essential as they may develop fresh lesions at any time. The relevant literature is discussed.