Abstract: Kawasaki disease (KD) is a childhood acute febrile illness with systemic vasculitis of an unknown etiology as the main pathological changes. KD often affects the coronary artery and, if not treated in time, the patients may develop coronary artery lesion (CAL). Currently, the standard treatment for the acute phase of KD are intravenous immunoglobulin (IVIG) and oral aspirin. Nevertheless, there are still 10% to 20% of patients unresponsive to IVIG, and risk of CAL was increased in these children. Literatures have reported a variety of therapeutic options for IVIG resistant KD, such as secondary IVIG therapy, glucocorticoid, infliximab, immunosuppressive agents, and plasma exchange. However, the outcomes of these therapies remain inconclusive. This article reviewed the progress in the treatment of IVIG resistant KD.