Kawasaki disease was described in 1967 by Tomisu Kawasaki. It affects children aged between one and 5 years, and it evolves with fever and small vessel vasculitis, which leads to cardiovascular complications, including coronary aneurisms, myocarditis, valve injuries, pericardial effusion and myocardial infarction; eventually involving many others organs. The etiology actually is not well known, as the exactly pathogenic mechanisms; however, now there are important advances. If the clinical signs and symptoms are identify early and the children received treatment with aspirin and intravenous immunoglobulin, the patients evolves without sequels. The Kawasaki disease is an infrequent disease in Mexico.