Congenital coronary fistula is a rare heart defect, which consists of a communication between a coronary artery and a cardiac chamber or coronary vessel. Most such fistulae drain into a right heart chamber or into the pulmonary artery. Congenital left coronary artery-left ventricle fistula is even more uncommon. In a search of the literature, we were able to find only 15 other cases of congenital left coronary artery-left ventricle fistula. We describe here the clinical picture of a patient with left circumflex coronary artery draining into the /eft ventricle, and the successful surgical repair of the lesion. As far as we know, such a case has never before been reported in detail.