Kawasaki disease, also known as mucocutaneous lymph node syndrome, is an immune-mediated systemic vasculitis syndrome and a relatively common pediatric febrile rash disease. To date, the etiology of Kawasaki disease is not entirely clear. Extensive epidemiological and clinical observations suggest that Kawasaki disease may be an acute immune dysregulation caused by infectious factors. Genetic factors are also related to the occurrence of Kawasaki disease. Various bacteria, viruses, mycoplasma, and their metabolic products, such as superantigens produced by Streptococcus and Staphylococcus, are also related to the development of Kawasaki disease.