Coronary artery fistula is a very rare congenital malformation with 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 one patient and one patient is still under observation. 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 9 patients, the fistulas originated from the left coronary system 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. Surgical closure of the fistula was successful in all operated patients [in one 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 procedure in the other patients. Neither hospital mortality nor severe complications occurred. Mean follow-up interval was 39.4 months and all patients except one were in NYHA I. In presence of symptoms of congestive heart failure, significant left-to-right shunt and arrhythmias, elective closure of coronary fistula is generally accepted, whereas indication is more controversial in asymptomatic patients.