Coronary artery fistula is a rare anomaly of the coronary arteries which results from an abnormal connection between a coronary artery with a cardiac chamber or vessel. Coronary steal phenomenon can lead to functional ischemia of the myocardium. The condition has been described extensively, however, the modalities for diagnosing this condition continue to advance.This review summarizes the approach to managing this uncommon condition.We discuss the various surgical options, including the technique involved in treating specific types of fistulas.We also review minimally-invasive options and trans-catheter closure along with indications for use of the different modalities.