Schwannoma of the hypoglossal nerve is extremely rare. We report the clinical manifestations of a patient with Schwannoma of the hypoglossal nerve with hemi-atrophy of the tongue and numbness in the lip. Magnetic resonance image study of the brain showed a lobulated mass at the right posterior fossa with an extension to the right upper neck. Surgical intervention was performed with right occipital craniotomy and a partial resection of C1 and occipital condyle. Pathological studies confirmed a Schwannoma with hemorrhages and necrosis.