Coronary artery anomalies occur either in isolation or in the context of congenital heart defects (CHD). Isolated coronary artery anomalies include anomalies of connection to the pulmonary artery or to the aorta, anomalies of the intrinsic coronary arterial anatomy including anomalous orifices, and anomalies of myocardial/coronary arterial interaction including myocardial bridges and fistulae. Such defects are of major significance in clinical cardiology and cardiac surgery because of their association with myocardial ischaemia and sudden death. Coronary anomalies associated with CHD can result from three types of developmental perturbation: (1) anomalous epicardial course (in congenitally corrected transposition of the great arteries and L-looped ventricles), (2) anomalous communication with a high-pressure ventricular cavity (pulmonary atresia with intact ventricular septum and hypoplastic left heart syndrome), or (3) anomalous connection to the aorta. Outflow tract defects represents 30–40% of CHD, and their main characteristic is great artery defects influencing coronary arterial anatomy.