We report a rare case of mitral stenosis associated with cardiac sarcoidosis and left ventricular aneurysm. A 60-year-old female was admitted with complaint of palpitation and dyspnea on exertion. Chest X-ray revealed cardiomegaly and pericardial calcification, but no bilateral hilar lymphadenopathy. Electrocardiogram showed various arrhythmia. Coronary angiography showed no stenosis in any coronary arteries. Cardioangiogram showed left ventricular aneurysm at the apex and mitral stenosis. Laboratory findings showed no evidence of sarcoidosis. Open mitral commissurotomy and left ventricular aneurysmectomy was performed. Pathological findings of the myocardium showed a remarkable degenerative change and a granulomatous inflammation. Postoperative biopsied specimens of right scalene lymph nodes revealed sarcoid reaction. The postoperative course was satisfactory, but arrhythmia remained. The patient was treated with steroids and pacemaker implantation. She has been doing well for 2 year postoperatively. An operative case of mitral stenosis associated with cardiac sarcoidosis and ventricular aneurysm due to sarcoidosis is very rare and the prognosis in patient is very poor usually.