Cardiovascular malformations are frequently observed in Turner's syndrome. Bicuspid aortic valve and coarctation of the aorta are commonly associated with Turner's syndrome whereas aortic dissection is rare but its rupture results in death. We experienced a case of ruptured dissecting aneurysm (Stanford type A) in a 30-year-old female with Turner's syndrome. Emergent total arch replacement was performed successfully. A literature review revealed 32 cases of aortic dissection in patients with Turner's syndrome, including 15 cases of rupture. However, survival after rupture was reported only two cases. To our knowledge, this report descries the third known case of successful surgical management of ruptured aortic dissection in Turner's syndrome.