Coronary artery fistula (CAF) is a rare congenital coronary artery anomaly and Infective endocarditis is a rare complication with high mortality rate. We present a 37-year-old female with a giant CAF that was complicated by infective endocarditis of the fistula with extension and involvement of the aortic valve. The diagnose was performed by transthoracic and three dimensional transesophageal echocardiography, then confirmed by computerized tomographic. The patient underwent successful aortic valve replacement and fistula repair after appropriate antibiotic therapy.