Disseminated intravascular coagulation (DIC) is an infrequent aortic dissection complication, and its optimal treatment remains controversial. A 55-year-old woman developed DIC associated with Stanford type B aortic dissection, which improved by administration of low-molecular-weight heparin combined with tranexamic acid, but the dissecting aneurysm of the descending aorta was dilated. After thoracic endovascular aortic repair for occlusion of entry tears detected by transesophageal echocardiography, DIC improved without anticoagulant therapy. Three months after treatment, the patient is doing well without complications. Endovascular repair is effective for DIC due to aortic dissection that requires anticoagulant therapy.