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Background: Ebsteins anamoly is a rare congenital disorder which is a common cause of congenital tricuspid regurgitation.Rheumatic mitral valve disease is common in developing countries but its association with Ebsteins anamoly is very uncommon. Case summary: We describe a 22 year old female with complaints of dyspnea and palpitations diagnosed with Ebsteins anamoly and rheumatic mitral stenosis. Discussion: The hemodynamic consequences and the interplay of these lesions are discussed.