A total of 110 children aged 3 months to 16 years underwent two-dimensional echocardiography of the coronary arteries. Forty-two normal subjects and 68 patients with Kawasaki disease were evaluated. All 68 patients with Kawasaki disease underwent selective coronary arteriography. The objectives of this study were to 1) develop a normal profile of the proximal left and right coronary arteries as to caliber and shape in infants, toddlers and children using echocardiography; 2) compare the dimensions and shape of the coronary arteries of patients with Kawasaki disease but no obvious aneurysms with those of the coronary arteries of normal children; and 3) develop criteria that would permit distinguishing a large but normal coronary artery from a true aneurysm in patients with Kawasaki disease. In the normal subjects and patients with Kawasaki disease, the caliber of the coronary arteries showed little variability from the ostium to 10 mm distally, and ranged in size from 2 mm in infants to 5 mm in teenagers. There was no significant difference between male and female subjects. The feature that distinguished the large but normal coronary artery without aneurysm from that with an aneurysm was its uniformity of caliber. Also, the caliber of the opposite coronary artery was generally at the lower limits of normal. It appears that the proximal coronary arteries of infants and children can be accurately assessed using high resolution two-dimensional echocardiography, and that sequential evaluation of subtle changes over time may be performed.