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The prevalence of coronary artery bypass grafting (CABG) based on the annual number of Kawasaki disease patients is 0.3–0.5% in Japan. The optimal time for performing CABG should be decided with understanding the characteristics of coronary arterial lesions due to Kawasaki disease and their natural history. CABG should only be performed when the native flow is severely impaired. Once good flow in the internal thoracic artery graft 1 year after surgery is confirmed, graft patency will persist for more than 20 years. The post-CABG left ventricular ejection fraction is related to the outcome in this population. At a minimum, good coronary revascularization to the left coronary artery can avoid a poor prognosis in these patients. Good coronary revascularization would improve the quality of life and the prognosis in this population.