Background Coronary artery fistula is a rare congenital malformation. Complications such as intracardiac shunts, endocarditis, myocardial infarction, aneurysm and sudden death can be observed. The purpose of this article is to present our experience with concomitant cardiac pathologies and discuss various therapeutic approaches including surgical and percutaneous intervention. Materials and methods During 18,272 diagnostic cardiac catheterizations, coronary artery fistulas were identified incidentally in 10 patients (0.05%). There were 3 female and 7 male patients. The patients’ ages ranged from 42 to 76 years. All patients with coronary artery fistula were preoperatively in New York Heart Association functional class and Canadian Cardiovascular Society class II or III. Results In addition to coronary artery fistula, coronary artery disease was detected in 4 patients (40%), a ventricular septal defect and an aneurysm of the sinuses of Valsalvae with aortic regurgitation in one patient (10%) and an anomalous origin of the LAD from the pulmonary trunk in one patient (10%) during cardiac catheterization. Four (40%) of the total of 10 patients had only coronary artery fistula. Surgical closure of the coronary artery fistula was performed in 7 patients (70%). An interventional fistula closure with a coil device was confirmed by cardiac catheterization in another 3 patients (30%). One patient of the latter group showed a small residual shunt from the LAD to the pulmonary trunk. No death or long-term morbidities could be observed. Conclusions Coronary artery fistulas are incidentally diagnosed during coronary artery angiographies in adults and should be closed to prevent complications.