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Abstract Coronary arteriovenous fistula is a rare entity and is mostly diagnosed and corrected in early childhood. We report the case of a 59-year-old female patient who was presented with chest pain during exertion for 3 months because of an aneurysmatic circumflex artery and coronary sinus fistula. We successfully ligated the circumflex artery close to the main stem and to the coronary sinus. The first marginal branch was revascularized by the left internal thoracic artery. The postoperative course was uneventful.