We report a patient with Miller Fisher syndrome who was treated with an intravenous high-dose of immunoglobulin. This syndrome is considered to be a benign variety of acute inflammatory demyelinating polyneuropathy (Guillain-Barré syndrome). However, there have been several reports of the need for ventilatory support and a few cases have had a fatal outcome. We observed a case of progressive Miller Fisher syndrome in a 3-year-old boy. Following 2 episodes of apnea lasting about 50 s each, he was treated with intravenous immunoglobulin (400 mg/kg/day) for 5 consecutive days. His respiratory state, general muscle strength, truncal ataxia and emotional state improved remarkably after this therapy.