Congenital coronary artery anomalies are rare but can carry a significant risk of myocardial ischemia, myocardial dysfunction, congestive heart failure, and sudden death. This risk seems to be highest during childhood and adolescence; therefore, an understanding of these anomalies is important for the pediatrician and pediatric cardiologist. This article focuses on the anatomic features, mode of presentation, diagnostic findings, and surgical treatments that are available for the two most common types of isolated congenital coronary anomalies that are associated with myocardial ischemia during childhood: (1) anomalous origin of a coronary artery from the opposite sinus of Valsalva with an interarterial course between the great arteries and (2) anomalous origin of a coronary artery from the pulmonary artery. A brief discussion of coronary artery fistulous connections completes the article.