A rare case of acute aortic dissection and aortic regurgitation in a patient with Turner's syndrome is reported. A 25-year-old woman with a history of Turner's syndrome presented to our hospital with complaints of chest pain and dyspnea. Emergent surgery was performed after chest roentgenography and two-dimensional echocardiography. Intraoperatively, the patient was found to have a single coronary artery and a bicuspid aortic valve. The patient was treated successfully with a composite graft consisting of a 24 mm woven dacron graft, a 21 mm St. Jude Medical aortic prosthetic valve, and an equine pericardial skirt. The composite graft was inverted into the ascending aorta and connected to a small coronary artery graft. The patient's postoperative course was uneventful. A cardiac catheterization was performed postoperatively and revealed a competent anastomotic site and no evidence of aortic regurgitation. We provide a brief review of coronary anomalies and aortic diseases associated with patients with Turner's syndrome. The most commonly associated complications include coarctation of the aorta and bicuspid aortic valve disease. The incidence of coronary anomalies in patients with Turner's syndrome is maybe rare.