Backgrounds: Coronary arteriovenous fistula (CAVF) is a rare congenital or acquired coronary artery anomaly in adult and pediatric populations and the long-term outcome is not fully known. We sought to evaluate the clinical and angiographic characteristics in patients with CAVF as well as clinical outcomes. Methods: We reviewed all patients (n=6820) presenting with CAVF between January 2000 and September 2007. Forty four Patients (0.65%, mean62.9짹12.8 years old, 19 males) had 60 CAVF by coronary angiography (CAG). We reviewed the clinical characteristics, clinical outcome and angiographic patterns of CAVF. Results: ST-T segment changes on ECG were seen in 16 patients (36.4%). There were 14 hypertensive (23.3%), 9 diabetics (20.5%), 9 smokers (20.5%), and 27 hyperlipidemics (62.8%). Significant coronary artery narrowing (stenosed more than 50%) was found in 12 patients (27.3%). Clinical symptoms of CVAF patient were chest pain (n=21), dyspnea (n=12), syncope (n=2), fatigue (n=1), palpitation (n=1), dizziness (n=2) and asymptomatic (n=5). The origin of the CAVF were left coronary artery (n=19), right coronary artery (n=9) and both coronaries artery (n=16). The most common drainage site of the CVAF was pulmonary artery (n=36, 60%), followed by left ventricle (n=12, 20%), right ventricle (n=3, 5%), left atrium (n=3, 5%), and right atrium, aorta, lung (n=2, 3.3%, respectively). Four patients underwent coil embolization and one patient underwent surgical ligation, whereas the remaining patients were medically treated. The majority of the study subjects were asymptomatic during mean 22.9짹19.1 months follow up period. There was no cardiac death or stroke. Conclusions: The prevalence of CVAF was 0.65%. The prognosis in patients with CVAF was excellent, although some patients needed invasive treatment.