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We present a case of 32-year-old female patient with rheumatic mitral stenosis with sinus venosus atrial septal defect (ASD) and anomalous drainage of the right superior pulmonary vein into right atrium. Sinus venosus ASD are uncommon and constitute 2-3% of interatrial communication. However, sinus venosus ASD with mitral stenosis is an extremely rare condition which was treated successfully by patch closure with left atrialization of superior pulmonary vein and mitral valve replacement.