The cause of Kawasaki disease (KD) is still unknown, but an infectious origin and genetic susceptibility have been suggested. The American Heart Association has changed the guidelines for diagnosis and treatment of KD in 2017. One goal is to better identify patients most at risk, particularly young children with « incomplete » KD. In case of intravenous immunoglobulin resistance, alternative treatments to the traditional use of corticosteroids, such as TNF-alpha inhibitors have been proposed. For all patients with coronary aneurisms, regular assessment of myocardial perfusion is recommended, either with stress echocardiography, MRI, scintigraphy or PET-scan.