Klinefelter`s syndrome which is characterized by hypogonadism with karyotype abnormality (47 XXY or 46 XY/47 XXY) in males has been reported to be associated with autoimmune diseases including rheumatoid arthritis and systemic lupus erythematosus. However, Klinefelter`s syndrome accompanying with polymyositis has rarely been reported. We report a case of KFS with polymyosits in a 38-year old man for the first time in Korea.