The coronary arteriovenous fistula (CAVF) is a rare congenital anomaly but constitutes the most common hemodynamically significant coronary artery anomaly. Transcatheter embolization is as an effective alternative to surgery even though procedure may be complicated by migration of the coil into peripheral vessels or pulmonary arteries. To our knowledge, the association of CAVF with Turner syndrome was not reported. We report a case of successful coil embolization of CAVF using a complex, helical-fibered platinum coil in patient with Turner phenotype with 46,XX.