A 7-year-old girl with ocular myasthenia gravis (MG) refractory to prednisolone and anticholinesterase compounds was treated with high-dose intravenous immunoglobulin (IVIg). Ocular symptoms disappeared rapidly by the administration of IVIg 300 mg/kg/day for five consecutive days. Booster infusions of IVIg at an interval of 2 weeks appeared to be effective in maintaining a remission at least as an adjunct to prednisolone. The levels of acetylcholine receptor (AchR) antibodies were not correlated with clinical improvement or aggravation. Thus, high-dose IVIg may be useful in refractory cases of MG, allowing a reduction in doses of immunosuppressive drugs.