Rheumatic mitral stenosis (MS) is one of the most common condition seen in the developing world. But the presence of mitral stenosis with severe symptoms at a very young age is rare. We report a case of an unusual association of a moderate perimembranous ventricular septal defect(VSD) with juvenile rheumatic mitral stenosis leading to severe pulmonary hypertension and heart failure in a 7 year old child. The association of congenital ASD with acquired Rheumatic MS ( LUTEMBACHER SYNDROME) is a well known entity. But, the association of a congenital VSD with rheumatic MS is very rare.