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ABSTRACT Kawasaki disease is a systemic vasculitis that mainly affects younger children. Although the definite cause still remains unknown but the clinical and epidemiologic findings discuss an infectious cause. The prevalence of incomplete Kawasaki disease reported 15 to 36.2%, and it is more frequent in the extremes of the age spectrum. Non delayed treatment of disease should be initiated because of critical cardiac vascular complications. Up to 15% - 25% of patients with Kawasaki disease who remain febrile after administration of first dose of intravenous immunoglobin plus aspirin are classified as refractory disease. These intravenous immunoglobin resistant cases are at increasing risk for coronary artery complications. The strategy on prediction of potentially non responder and treatment of intravenous immunoglobin resistant patients is now controversial but some useful points were recommended.