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Cor triatriatum sinister is an extremely rare congenital heart defect. Very few cases have been reported for cor triatriatum associated with mitral regurgitation. We are reporting an exceptional case of cor triatriatum associated with calcified mitral valve resulting mitral stenosis. Preoperatively, mitral stenosis was underestimated due to the presence of restricted communication between two chambers of the left atrium. The patient was operated for cor triatriatum repair with mitral valve replacement.