Kawasaki disease is a leading cause of acquired heart disease in children in the USA. An acute vasculitis of unknown etiology, it occurs predominantly in infancy and early childhood, and more rarely in teenagers. Coronary artery aneurysms or ectasia develop in approximately 15-25% of children with the disease. Treatment with intravenous gamma globulin, 2 g per kg, in the acute phase reduces this risk three- to fivefold. Angiographic resolution occurs in approximately one-half of aneurysmal arterial segments, but these show persistent histologic and functional abnormalities. The remainder continue to be aneurysmal, often with development of progressive stenosis or occlusion. The worst prognosis occurs in children with so-called 'giant aneurysms', i.e. those with a maximum diameter greater than 8 mm, because thrombosis is promoted both by sluggish blood flow within the massively dilated vascular space and by the frequent development of stenotic lesions. Serial stress tests with myocardial imaging are mandatory in the management of patients with Kawasaki disease and significant coronary artery disease to determine the need for coronary angiography and transcatheter interventions or coronary bypass surgery. Continued long-term surveillance in patients with and without detected coronary abnormalities is necessary to determine the natural history of Kawasaki disease.