Coronary artery fistulas represent the most common hemodynamically significant congenital defect of the coronary arteries and the clinical presentation is mainly dependent on the severity of the left-to-right shunt. We describe a case of a 55-year-old man with history of chest pain and without history of previous significant chest wall trauma or any invasive cardiac procedures. A coronary multislice computed tomography showed two large coronary fistulas arising from the left anterior descending coronary artery and ending in an angiomatous plexus draining into the common pulmonary trunk. Coronary angiography confirmed the CT finding and showed a third fistulous communication arising from the sinus node artery.Although coronary fistulas are infrequent, they are becoming increasingly important because their management and treatment could prevent serious complications. The latest guidelines of the American College of Cardiology/American Heart Association indicate as Class I recommendation the percutaneous or surgical closure for large fistulas regardless of symptoms.In this manuscript, we provide a detailed review of the literature on this topic, focusing on the clinical management of these patients.