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Coronary artery fistula (CAF) is defined as a direct communication of a coronary artery with a cardiac chamber, great vessel or other vascular structure, bypassing the myocardial capillary bed. Congenital CAFs joining into the pulmonary artery are rare cardiac anomalies. CAFs arising from two coronary arteries are even more rare especially when combined with valvular heart disease. The coincidence of CAFs with aortic insufficiency is relatively rare and sometimes might cause myocardial ischemia.We present a case of bilateral coronary-pulmonary artery fistula combined with severe aortic insufficiency causing myocardial ischemia and who subsequently underwent fistula ligation during aortic valve surgery.