Kawasaki Syndrome is a newly recognized clinical entity characterized by multisystem involvement. It has an acute onset and a triphasic clinical course. Although essentially a self-limited disease, permanent vascular damage, especially involving the coronary arteries, may result. Pathologically the disease is characterized by widespread vasculitis. There is a monomodal age distribution with peak occurrence during the first 2 years of life; few affected over the age of 8 years. Males outnumber females 1.5:1, persons of Japanese extraction are overrepresented compared with other races, and Caucasians are underrepresented. Community-wide epidemics occur in diverse locations but there is no evidence for direct person-to-person transmission. Etiology remains unknown. Therapy remains supportive and should be directed at careful clinical evaluation for cardiovascular abnormalities and antiplatelet aggregation therapy.