Kawasaki disease is one of the most common vasculitides in childhood, predominantly affecting children less than 5 years of age. The disease often presents with a unique collection of signs and symptoms that include prolonged fever, bilateral nonexudative conjunctivitis, mucosal changes, and cervical lymphadenopathy, rash and extremity changes. Although changes in laboratory investigations are often present, none have been found to be specific for the disease. The diagnosis is based on the clinical criteria established by the American Heart Association, which requires the presence of fever for at least 5 days in addition to at least 4 principal clinical features. Although the acute phase of the disease is self-limited, coronary artery aneurysms and other serious cardiovascular sequelae may develop later, including a considerable proportion of those not appropriately treated. Intravenous immunoglobulin, usually given with acetylsalicylic acid, is the treatment of choice and has been proven to be highly effective in reducing the prevalence of coronary artery aneurysms in this population.