Coronary artery anomalies are commonly associated with congenital heart disease. It is important to recognize these anomalies to avoid errors in diagnosis, decrease technical difficulties in angiography, and prevent inadvertent injury in surgery. We found that of 66,884 patients who underwent coronary arteriography between 1972 and 1982 at the Cleveland Clinic Foundation, 1,000 had coronary anomalies. Of these, 101 had associated congenital anomalies: 29 had mitral valve prolapse, 18 had bicuspid aortic valves, 16 had tetralogy of Fallot, 11 had corrected transposition, 10 had a univentricular heart, 6 had coarctation of the aorta, 3 had ventricular septal defects, and 8 had miscellaneous congenital heart defects. The most common coronary anomaly was ectopic origin of a coronary artery: 30 from the sinus of Valsalva, 12 from the ascending aorta, 11 from the pulmonary artery. Nineteen patients had no left main trunk. Thirteen patients had coronary artery fistulas and 21 had miscellaneous coronary anomalies.