Kawasaki disease is an acute illness of early childhood primarily affecting coronary arteries accompanied by aneurysms. In his first report of 50 patients in Japanese in 1967, Dr. Tomisaku Kawasaki suggested interesting immunological abnormalities of Kawasaki disease, occasional appearances of autoantibodies and proliferations of reticuloendothelial cells in cervical lymph nodes. This review briefly summarizes the aberrations in the immune systems of patients with Kawasaki disease, in antibody production and in responses of macrophage and monocyte.