Isolated single coronary artery is a rare congenital anomaly occuring in approximately 0.024% of the population. This entity can be diagnosed during life only by coronary angiography. Ten patients with isolated single coronary artery are reported. Based on angiographic analysis, a new classification is proposed, according to the site of origin and anatomical distribution of the branches. Typical angina did not occur with single coronary artery in the absence of coexisting coronary artery disease or aortic stenosis. No correlation was apparent between the type of anomalous patterns and the symptoms of angina.