Kawasaki disease(KD) is characterized with acute systemic vasculitis, occurs predominantly in children between 6 months to 5 years of age. Patients with thisdiseaserecover well and thediseaseis self-limited in most cases. Since it can lead to devastating cardiovascular complications, KD needs special attention.Recentreports show steady increases in the prevalence of KD in both Japan and Korea. However, specific pathogens have yet to be found.Recentadvancesin research on KD include searches for genetic susceptibility related to KD and research on immunopathogenesis based on innate and acquired immunity. Also, search for etiopathogenesis and treatment of KD has been actively sought after using animal models. In this paper, therecentprogress of research on KD was discussed.