Congenital heart disease is related to events occurring in the embryonal stage, while rheumatic heart disease is a sequelae of immune-mediated damage following streptococcal infection. Rheumatic heart disease is a well-known entity in developing world. Congenital heart disease is also common cause of hospital admission. However, coexistence of rheumatic heart disease with congenital heart disease occur rarely. Here we report an unusual case of rheumatic mitral stenosis with restrictive patent ductus arteriosus (PDA) and severe pulmonary arterial hypertension leading to Eisenmenger syndrome in a 13 years old girl. This case highlights the need for careful examination for coexisting rheumatic disease and congenital heart disease. Eisenmenger syndrome could be a presentation of juvenile severe rheumatic mitral stenosis when it is associated with congenital shunt lesion like PDA