Anomalous origin of a coronary artery from the pulmonary artery (PA) is an unusual abnormality. These anomalies are often hemodynamically significant and can lead to myocardial ischemia, myocardial infarction, and sudden cardiac death. Anomalous origin of the left coronary artery (LCA) from the pulmonary trunk is known as Bland-White-Garland syndrome and causes death in 80% to 85% of affected children in the first year of life. Treatment of the LCA arising from the PA is typically surgical, with ligation and reimplantation of the LCA to the aorta. In contrast, origination of the right coronary artery from the PA is usually a benign anomaly, although clinically significant sequelae can develop. Controversy exists regarding the need to intervene in the cases of patients with this usually incidentally discovered anomaly. We review the clinical presentation and treatment of patients with each anomaly and the current recommendations regarding the clinical course and management of such patients.