A 39-year-old man was admitted to our hospital because of atrophy of the right side of the tongue of 6 years' duration. Neurological examination showed atrophy and fasciculations in the right side of the tongue. Magnetic resonance imaging showed atrophy and high signal intensity areas on T1 and T2 weighted images in the right masseter, temporal, lateral and medial pterygoid muscles. Electromyography showed fibrillations and positive sharp waves at rest, high amplitude polyphasic motor unit potentials with weak contraction in the right tongue and masseter muscles. Examination of autonomic functions suggested postganglionic lesion of right facial sympathetic nerves. We believe that this case may be related to progressive facial hemiatrophy.