A coronary artery fistula between a coronary artery and a cardiac chamber is a rare condition. We reported a case of right coronary artery fistula to the left ventricle in a 57-year-old man who had 2-year history of chest pain and exercise dyspnea without significant coronary atherosclerosis with abnormal left ventricular size and function. It was important to recognize this anomaly and our experience showed that transcatheter occlusion of coronary artery fistula was a safe and effective procedure in the presence of symptoms of congestive heart failure, significant left-to-right shunt or refractory to medical treatment.