Neurinoma originates from any nerve covered with a Schwann cell sheath and can occur in any cranial, sympathetic, or peripheral nerve. Hypoglossal neurinomas are rare and most of them are intracranial, but they may extend extracranially. Most intracranial neurinoma arise from the sensory division of cranial nerve but a motor nerve such as hypoglossal nerve is rarely involved. Although the typical sign of hypoglossal neurinoma is ipsilateral hemiatrophy of the tongue, it is easily overlooked. For the diagnosis of hypoglossal nerve tumor, CT scanning with contrast enhancement and MRI should be included, and they are greatly aids in planning the radical removal of the tumor. We experienced a case of intracranial hypoglossal neurinoma with extracranial extension in a 43-year-old woman. The patient showed otherwise unremarkable except 4 months history of right infraauricular mass and right tongue hemiatrophy. Computed tomography and magnetic resonance imaging for local diagnosis was valuable and we could remove the mass by one stage operation via suboccipital transcervical approach.