Coronary artery fistula is a very rare congenital malformation with an abnormal coronary-cameral communication that may involve any chamber and any or all coronary artery branches. We present our experience with 11 consecutive patients (mean age 16.6 years, ranging from 4 to 64 years); 9 of them were treated surgically, spontaneous closure of the fistula was observed in 1 patient and 1 patient is still under observation. Nine patients were under 17 years of age at the time of operation whereas only 2 patients were older (56 and 64 years). Fistulas originated from the right coronary artery in 4 patients and drained either into the right (n = 3) or into the left system (n = 1). In 8 patients, the origin of the coronary artery from the aorta was normal and the fistulous communication developed with the right cardiac structures only. In 2 patients, both coronary arteries were involved in the pathological drainage and 2 patients were demonstrated to have multiple drainage from the left coronary artery. Additional congenital cardiac malformations were found in 2 patients: severe tricuspid valve regurgitation in 1 and ventricular septal defect in another patient. Surgical closure of the fistula was successful in all operated patients (in 1 case treatment was possible without cardiopulmonary bypass). Simple ligation of the fistula was performed in 1 patient, intracardiac closure of the fistula was combined with different reconstructive procedures in the other patients. No hospital mortality nor severe complications occurred in this small group of patients. The mean follow-up interval was 39.4 months and all patients were in NYHA functional class I, except 1 with moderate tricuspid and mitral valve regurgitation. In the presence of symptoms of congestive heart failure, significant left-to-right shunt and arrhythmias, elective closure of coronary fistula is generally accepted, whereas the indication is more controversial in asymptomatic patients. Considering the low perioperative morbidity, we recommend surgical closure of coronary fistulas with significant shunt and/or increased coronary artery diameter.