KS is a fascinating illness of childhood that has emerged over the last 30 years and is now recognized as the most common cause of acquired heart disease in children in the United States. It has a dramatic clinical presentation in most cases, but for a small number of cases that present with a more subtle disease there appears to be a greater risk of coronary artery complications. Progress has been made in the management of KS, but although the disease appears to be related to an infectious etiology, no infectious agent has been proven to be the cause. Finally, issues in long-term management and prognosis are yet to be clarified.