The etiology of Kawasaki disease (KD) remains unknown, although a number of epidemiological and clinical observations may suggest that it is triggered by a single or more possibly multiple infectious agents, each of which can result in the clinical manifestation of the disease. Advances have been made in the management of the disease with the introduction of aspirin and IVIG that have had a significant impact on lowering the rate of coronary artery aneurysms and death from the disease. Questions remain regarding the management of those patients who fail to respond to IVIG. It appears that some patients with severe KD who are resistant to IVIG may benefit from IV pulse steroids therapy or infliximab infusion. However, a recent multi-center, randomized-controlled trial do not support for the addition of a pulsed dose of intravenous methylprednisolone to the conventional IVIG therapy for the primary treatment of KD. It still remains to be seen whether other anti-inflammatory agents such as immunosuppressive therapies, or new biologics play a role in the management of patients with KD.