Introduction: Congenital coronary artery fistula is a rare congenital anomaly and can be complicated by endocarditis, myocardial ischemia or rupture. But its clinical significance and natural course are still unclear. The purpose of this study is to review a 13 years experience with congenital coronary artery fistula and to discuss the effective management. Material and Method: From March 1996 to February 2009, 19 patients underwent operation or transcatheter closure of cornary artery fistula. We reviewed their medical records and analyzed the clinical characteristics and outcomes. Result: All patients had systolic or continuous murmur. Initially only 3 patients had heart failure symptoms. The mean Qp/Qs was 1.30 짹 0.32 and the median follow up period is 66.03 짹 41.40 months. Nine fistulas originated from the right coronary artery, nine from the left and one from both coronary arteries. Drainage was to the right ventricle (11), right atrium (4), left ventricle (3) and pulmonary artery (1). 11 patients had an operation, 7 patients had a transcatheter closure and one patient underwent both procedures. After transcatheter closure, two cases had residual shunt and thrombus observed in one patient. One patient had a reoperation due to perforation of mitral valve. There was no significant difference of clinical status between operation group and intervention group. There was no mortality associated with operation or catheter procedure. All of them have no evidence of myocardial ischemia during follow up periods. Conclusion: Transcatheter closure has some limitations on the treatment of coronary artery fistula. However that is still reasonable option for accurate diagnosis and effective management of coronary artery fistula.