Abstract Intravenous immunoglobulin is effective to resolve inflammation of Kawasaki disease and reduce the incidence of coronary artery abnormalities. However approximately 20% of patients with Kawasaki disease had persistent or recurrent fevers after intravenous immunoglobulin and are considered to have a high risk for coronary artery abnormalities. Recently, we developed a new risk score that resistance to intravenous immunoglobulin could be identified with high sensitivity and specificity in advance using seven laboratory and demographic variables available before initiation of primary therapy. In this article, we will review prediction of intravenous immunoglobulin unresponsiveness. In addition, we will focus on the risk stratification of primary therapy using the risk score.