We report a 70-year-old patient who presented with a 4-year history of weakness of paravertebral muscles. Electrodiagnostic studies revealed a mixed neurogenic-myopathic pattern. Light microscopic examination revealed atrophic fibers with rimmed vacuoles; electron microscopy demonstrated cytoplasmic and intranuclear filaments measuring about 16 nm in width, consistent with the diagnosis of inclusion body myositis. Therapy with corticosteroids provided only a mild and transient benefit. Ten months after the initial evaluation, clinical and electrodiagnostic examination demonstrated mild progression of the disease.