Schwannomas of the trochlear nerve are very rare. Only 25 cases without associated neurofibromatosis were reported in the literature, only 15 of which were surgically verified. We report an unusual case of a 31-year-old man who presented with isolated unilateral trochlear nerve palsy due to a left sided trochlear nerve schwannoma. The tumor was totally resected without additional morbidity using an infratentorial lateral supracerebellar approach. (C) 2002 Elsevier Science Ltd. All rights reserved.