A congenital coronary artery fistula is an uncommon anomaly which has a direct communication between a coronary artery and the lumen of any one of the four cardiac chambers, or the coronary sinus, or its tributary veins or the superior vena cava. The right coronary artery is involved most frequently, and the abnormal communication in most often is to the right ventricle followed in incidence by drainage into the right atrium and the pulmonary artery. Recently. we experienced a case of congenital coronary artery fistula associated with valvular heart disease. The fistulous communication was noted between the left circumflex artery and the left atrial appendage. Under the cardiopulmonary bypass, the internal obliteration of the left atrial appendage, mitral valve replacement, and aortic valve exploration were accomplished. Postoperative hospital course was uneventful and the patient was discharged without any problems.