To determine the accuracy of two-dimensional echocardiography in the identification of congenital anomalies of the aorta, we compared two-dimensional echocardiographic with angiographic results in 261 consecutive infants and children with congenital heart disease (age 1 day to 20 years, mean 3.3 years). Two-dimensional echocardiography was performed and interpreted without knowledge of angiographic results. Complete visualization of the ascending and descending aorta and aortic arch branches was possible by two-dimensional echocardiographic examination in suprasternal, parasternal, and subcostal views of 255 patients (98%). Identification of the esophagus during swallowing aided the diagnosis of anatomic characteristics of aortic arch. One or more significant aortic arch anomalies were present on angiograms of 116 of 255 patients (46%) and were detected by two-dimensional echocardiography in 110 (sensitivity 95%, 99% specificity). Anomalies detected by two-dimensional echocardiography/angiography were ascending aorta hypoplasia in four/four, truncus arteriosus three/three, right aortic arch 31/31, anomalous subclavian artery 11/16, coarctation 27/29, and patent ductus arteriosus 53/57. We conclude that two-dimensional echocardiography can be used to determine the anatomy of the aorta in most infants and children. In selected patients, two-dimensional echocardiography may eliminate the need for angiographic examination before surgery for congenital heart disease.