Kawasaki disease, or mucocutaneous lymph node syndrome, is the leading cause of acquired heart disease in children in the United States and other developed countries. Coronary artery lesions are the most significant cause of morbidity and mortality. Treatment should ideally be provided within 10 days of symptom onset to reduce the risk of coronary artery complications. The standard of care for treatment is intravenous immunoglobulin plus aspirin, but adding corticosteroids may provide additional benefit for high-risk patients. Some patients do not respond to intravenous immunoglobulin and require additional therapy. Part II of this continuing medical education article will focus on the complications of Kawasaki disease and potential treatment options.