Congenital and acquired coronary artery anomalies are associated with significant morbidity and mortality and can be sudden in onset. The spectrum of congenital lesions include anomalous origin from the pulmonary artery, critical left main stenosis/atresia, coronary artery fistulas, anomalous aortic origin and intramyocardial courses. The spectrum of acquired lesions include Kawasaki disease, late postoperative obstructions in patients who had coronary artery surgical manipulations and iatrogenic injuries that can occur in the catheterization laboratory or the operating room. Surgical therapies for ischemic syndromes associated over the long term of these anomalies are presented herein.