A neurilemmoma is characteristically a capsulated benign tumor of nerve sheath origin in cranial, sympathetic and peripheral nerves. Although it may arise from any nerve possessing a sheath of Schwann and is a relatively common tumor on the any other region, reports of neurilemmoma of the external ear canal are very rare. We have recently experienced a rare entity of neurilemmoma of the external ear canal. The patient was a four year old boy who had noticed gradual swelling of superior-posterior portion of the ear canal orifice for a few months of duration. This particular case is of considerable interest not only because of tumor's location but also because of its large size.