Anomalous origin of a coronary artery from the aorta is a potentially serious anomaly that occurs in about 0.1-0.2% of the population. This percentage is small; however, it translates into about 4000 annual births with these anomalies. The clinical presentation of these anomalies is rare, and hence most are and will remain asymptomatic. The various anatomic anomalies are described, with anomalous origin of the left coronary artery that then passes between the aorta and pulmonary artery being the most serious of these anomalies. The pathophysiology resulting from these anomalies is described, as are methods for identifying those who require treatment; however, we still do not know the best methods of determining which patients need treatment.