Congenital anomalies of the coronary vasculature are usually asymptomatic and rare with an incidence of less than 1% and there are few cases reported of congenital absence of the left circumflex artery. It is important to recognize this anomaly and define the anatomy by left heart catheterization or coronary angiography by computerized tomography when considering percutaneous intervention or coronary artery bypass surgery on patients without a left circumflex artery.