The case was a 27-year-old woman with a history of Turner syndrome. The patient underwent ascending-descending aorta bypass for aortic coarctation 6 years previously and underwent subsequent follow-up on an outpatient basis. She consulted our department because of fever, chest pain and headache as the main complaints. Age-indeterminate type A aortic dissection was found on computed tomography, and she was admitted to the hospital on the same day. Echocardiography also revealed an enlarged aortic root and bicuspid aortic valve. Aortic root replacement and total arch replacement were performed, and her postoperative course was favorable. It is reported that in cases of Turner syndrome with aortic coarctation, aortic aneurysm and aortic dissection are likely to occur due to the vulnerability of the aortic wall. We encountered a patient with Turner syndrome who underwent ascending-descending aorta bypass for adult aortic coarctation and subsequently developed type A aortic dissection, underwent aortic root and total arch replacement, and rehabilitated after surgery, as well as provide bibliographic considerations.