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Direct coronary artery reimplantation to the aorta and mitral valve repair were successfully performed in a 29-year-old female with Bland-White-Garland syndrome (BWG syndrome). Under cardiopulmonary bypass, the main pulmonary artery was completely transected and the left coronary artery was excised with a cuff of pulmonary artery wall. Then the left coronary artery was directly anastomosed to the ascending aorta. Mitral regurgitation was repaired with valvulo-annuloplasty. The post operative course was excellent.