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Kawasaki disease (KD) is a multisystem inflammatory illness of infants and young children that could result in acute vasculitis. Death due to this systemic vasculitis syndrome most frequently results from thrombosed coronary artery aneurysms and coronary arteritis. Without treatment, 25% of children with KD develop coronary artery abnormalities. Current therapy for KD consists of intravenous immunoglobulin within the first 10 days of fever onset; this treatment reduces the prevalence of coronary artery abnormalities to 5%. Advances in genetic and proteomic analysis have sparked a worldwide effort to identify genes and potential biomarkers associated with KD. In this review, we highlight important research advances that have been made in the epidemiology, etiology, genetic polymorphisms, diagnosis, and therapy of KD.