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Anti-mitochondrial antibody type 2 is a diagnostic marker of primary biliary cirrhosis and complicates myositis. Myositis with anti-mitochondrial antibody type 2 is clinically characterized by slowly progressive limb, cardiac, and respiratory muscle weakness as well as serum creatinine kinase elevations. However, there has been few cases with eye symptoms. We herein report a 59-year-old woman with anti-mitochondrial antibody type 2 who presented with diplopia and ptosis. Magnetic resonance imaging revealed bilateral o