Abstract
Coronary artery fistula is a rare congenital malformation of high variability. The disease is illustrated with a description of a case example. The management of patients with coronary artery fistulas remains controversial. Both spontaneous regression and life threatening complications have been described. The fistula can be ligated or embolised; however, there are no long term outcome data regarding management. Intraoperative risk of myocardial infarction is less than 5% and death rate varies between 0% and 6%. Due to a small number of cases being described in the literature and a lack of evidence on optimal management, further research is needed in order to determine the best treatment options.