Kawasaki disease is the leading cause of acquired heart disease in children in many parts of the world. It is an acute systemic vasculitis of unknown etiology predominantly affecting children under 5 years of age. It is diagnosed clinically by the presence of a constellation of manifestations and exclusion of other diseases with similar findings. Children develop a spectrum of cardiovascular manifestations including coronary artery involvement in 30-35% of untreated cases with myocardial infarction and death in < or = 2% of them. Acute stage treatment is by administration of intravenous immunoglobulin and aspirin. Those with coronary artery involvement need long-term cardiac risk stratification and management because many of them will become the part of a growing pool of adults with ischemic heart disease.