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Objective: To report a case of neuritis like optic neuropathy accompanied with mtDNA G11778A mutation with long-term follow-up.Methods: Case report.Results: A boy of 10 years old presented with a complaint of second vision loss in the right eye for one day.He had a sudden vision loss in the same eye one month ago, and was diagnosed as "optic neuritis".His visual acuity recovered to 1.0 soon after intravenous methylprednisolone pulse therapy which was followed by tapering oral methylprednisolone.He had a past medical history of thrombocytopenic purpura two years ago which was already cured.