Kawasaki disease is an acute self-limited vasculitis of infancy and early hildhood that is now the leading cause of acquired heart disease in children in the United States and Japan. An infectious cause is suspected, although the etiology remains unknown. Children typically are seen with the acute onset of fever followed by signs of mucosal inflammation and vasodilatation that evolve over the first week of the illness. Laboratory tests reveal a marked systemic inflammatory response. If untreated, 1 in 4 children will develop permanent damage to the coronary arteries. Intravenous gamma globulin in conjunction with aspirin is effective in reducing the inflammation and preventing coronary artery abnormalities if administered within the first 10 days of illness. This poses a difficult situation for the pediatric care provider who must distinguish Kawasaki disease from other rash/fever syndromes in a timely manner so that appropriate therapy can be initiated.