We report a 74-year-old woman with anit-Golgi antibody and anti-SS-A/Ro antibody who contracted inflammatory myopahy presenting 'ALS-like' symptoms. We identified anti-Golgi antibody directly using confocal microscopy and successfully treated her with steroid. This report suggests that there is a new categorized subgroup of inflammatory myopathy with these specific antibodies and the pattern of autoantibody in these patients indicates the specific clinical course and treatment strategy.