Hypoglossal neurilemmoma is extremely rare. Intracranial hypoglossal neurilemmoma has been reported to the present most commonly as a space-occupying lesion with symptoms of raised intracranial pressure. A 68-year-old women presented with deviation of the tongue to the left on protrusion. Preoperative radiological images revealed an extra-axial mass in and around the hypoglossal canal. The tumor was totally resected via retrosigmoid suboccipital approach with burrhole craniectomy. Histopathological examination verified a neurilemmoma. She had no neurologic abnormality except hypoglossal palsy which recovered completely in six months. Retrosigmoid suboccipital approach with burrhole craniectomy can be an useful approach in intracranial hypoglossal neurilemmoma without extracranial extension or with minimal extracranial extension into the hypoglossal canal.