Hypoglossal schwannoma is a rarely encountered skull base lesion that has generally both intracranial and extracranial parts. This article reports a case of hypoglossal schwannoma that is purely extracranial and presents itself only with persistent headache. Magnetic resonance imaging delineated a mass at the skull base that consisted of both cystic and solid components, consistent with a nerve sheath tumor. Cervical approach was chosen, and the mass was completely removed. Postoperatively, the patient developed hypoglossal nerve palsy because the hypoglossal nerve was sacrificed. Hypoglossal schwannomas are briefly discussed with a literature review.