Kawasaki disease is a generalized vasculitis of unknown etiology affecting children. Although self-limited in most cases, the disease can cause coronary artery abnormalities, including coronary aneurysms. These are more frequently seen in untreated children but can also occur in those who received appropriate therapy at the time of diagnosis. The coronary artery abnormalities can resolve or persist. Those that persist can lead to coronary stenosis, thrombotic occlusion, or rarely, rupture, resulting in myocardial infarction, the primary cause of death in these children. Affected patients require close follow up for cardiac surveillance, which frequently involves the need for advanced cardiovascular imaging studies. Given their young age, sedation or general anesthesia is required in most cases. Management requires a thorough understanding of the pathophysiology of the disease process, particularly the cardiac manifestations of the condition, along with knowledge of the effects of the various anesthetic agents on hemodynamics and ventricular function.