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Kawasaki disease was first described in Japanese in 1967, and the first English language report appeared in 1974. Consequently, only recently have Kawasaki disease patients reached adulthood and come to the attention of adult cardiologists. As children with Kawasaki disease grow up, adult cardiologists are likely to see increasing numbers of these patients with cardiovascular complications. The purpose of this review is to highlight recent advances in our understanding of the late cardiac sequelae of Kawasaki disease.Patients with persistent or remodeled coronary aneurysms after Kawasaki disease have a high rate of complications including thrombosis or stenosis leading to myocardial infarction. Whether patients with Kawasaki disease are at risk of accelerated atherosclerosis remains controversial, but there may be persistent inflammation in the arterial wall of coronary aneurysms long after Kawasaki disease, and myofibroblasts likely play a central role in the arterial remodeling process.The vasculopathy of Kawasaki disease appears to be distinct from that of atherosclerosis, and optimal management strategies for the two conditions differ. Patients with persistent or remodeled coronary aneurysms or regressed aneurysms after Kawasaki disease are at increased risk and require long-term follow-up by cardiologists knowledgeable about management issues in this patient population.