Coronary arteriovenous fistula is a rare congenital coronary artery anomaly in adults. Most such fistula drain into a right heart chamber or into the pulmonary artery. Congenital left coronary artery-left ventricle fistula is even more uncommon. Aortography and selective coronary angiography are still the diagnostic mode of choice. However, recent studies have demonstrated that two-dimensional echocardiography with or without Doppler color flow imaging is a useful noninvasive tool in the diagnosis of coronary artery fistula. A 29 year-old female who had an exertional dyspnea, chest pain, and continuous diastolic murmur was referred to our hospital for further evaluation. The transthoracic two-dimensional and color Doppler echocardiography revealed a dilated left coronary artery which was drained into left ventricle. The coronary angiography showed that the left circumflex artery was very dilated and tortuous, and contrast medium passed from the left circumflex artery into the left ventricular cavity via a fistula. However, the right coronary artery and the left anterior descending artery were normal. We reported a case of an echocardiographically documented fistula between the left circumflex coronary artery and the left ventricle in young woman.