Abstract
Olfactory groove schwannomas are extremely uncommon and less than 30 cases are reported in the literature. We report a 21-year-old developmentally-retarded boy who experienced severe headache and aggressive behaviour for 5 months. Imaging showed a cystic mass in the subfrontal region, which was removed by craniotomy. The lesion had a vascular supply from the anterior ethmoidal arteries and it was noted to be attached to the right olfactory nerve. It was removed completely and histology showed it to be a schwannoma. Olfactory groove schwannomas are rare lesions and should be differentiated from meningiomas, neuroblastomas and dural-based metastatic lesions of the anterior cranial base.