BackgroundCongenital left main coronary atresia is an extremely rare coronary anomaly. Long-term surgical outcomes and the optimal management strategies for recurrence of ischemia remain uncertain. Herein, we present a case involving successful redo coronary artery bypass grafting for unstable angina 27 years after the initial coronary artery bypass grafting for congenital left main coronary atresia.Case presentationA 33-year-old woman was referred to our department with unstable angina. At the age of 6, she had undergone coronary artery bypass grafting of the second diagonal branch using the left internal thoracic artery and the obtuse marginal branch using saphenous vein grafting for left main coronary atresia. Although a coronary angiogram showed a patent left internal thoracic artery graft to the second diagonal branch and a patent saphenous vein graft to the obtuse marginal branch, the left anterior descending artery was not being perfused by the grafts because of a disruption of blood flow to the left anterior descending artery from the left internal thoracic artery. Therefore, we performed a redo coronary artery bypass grafting using the in situ right internal thoracic artery to the first diagonal branch, which was to be connected to the left anterior descending artery, resulting in amelioration of the ischemia of the left anterior wall. The patient was discharged 10 days after the operation and has been in good health for over 3 years without recurrence of chest symptoms.ConclusionsCoronary revascularization using a saphenous vein and left internal thoracic artery grafts is effective in achieving an adequate blood supply to the distal coronary arteries, and this effect can last for decades. However, careful follow-up is necessary because recurrent myocardial ischemia due to the development of a coronary artery occlusion may occur in adulthood.