We discdbed a case of a 55-yr-old woman diagnosed with Lutembacher syndrome and rheumatic pulmonary stenosis.Congenital atrial septal defect was found in age 7 and rheumatic fever in age 34.As the patient developed pulmonary hypertension with calcified mitral valve leaflet and pulmonary stenosis so surgery was not indicated.So the patient was managed by medical therapy alone.