We report the case of a patient with oral lesions on the upper surface of his tongue. Intraoral examination revealed marked right-sided hemiatrophy of the tongue with fasciculation, partial deviation of the tongue on the right side, and inability of the patient to completely deviate the tongue toward the left side of his mouth on protrusion. A magnetic resonance image showed presence of a tumor lesion characterized by irregular margins localized in the intradural space, with a lateral extension along the omolateral hypoglossal canal. This was consistent with the diagnosis of a hypoglossal neurinoma, later confirmed by the histopathologic examination.