Kawasaki disease (KD) is one of the cytokine-associated diseases, which is systemic inflammatory response syndrome. Tumor necrosis factor-alpha(TNF-alpha), as proinflammatory cytokine, plays an important role in the vascular injury of KD. Nuclear factor kappa B (NF-kappaB) is a pivotal transcription factor for genes that encode the proinflammatory cytokines, chemokines and adhesion molecules that mediate inflammation. We have already reported the activation of NF-kappaB in peripheral blood mononuclear cells with the dominant of monocytes/macrophages. There is ample evidence of a central role of peripheral blood monocytes/macrophages during acute KD. The standard therapy of KD is intravenous immunoglobulin (IVIG). According to circumstances, corticosteroid has been used to treat the KD patients. Recently, it has been reported that new biologic therapy, such as anti-TNF agents are useful for the patients who failed to respond to an initial IVIG. We focus on the different mechanism of IVIG, corticosteroid and anti-TNF agents with regard to the anti-inflammatory effects.