Congenital coronary arteriovenous fistula is a ~are condition, and with widespread use of cardiac catheterization, angiography and selective coronary arteriography is being recognized with increasing frequency. Surgical correction is strongly recommended to prevent the development of congestive heart failure,angina, subacute bacterial endocarditis, myocardial infarction, and pulmonary hypertension, as well as coronary aneurysm formation with subsequent rupture or embolization. I report a case of congenital coronary arteriovenous fistula of the left main coronary artery to the fight atrium in a 23 year old female, which is associated with bacterial endocarditis with right atrial vegetation.