A 43-year-old pregnant woman was transferred to our hospital at 30 weeks' gestation for evaluation of chest and back pain. The patient was diagnosed with Stanford type A acute aortic dissection and had a family history of aortic dissection, with physical findings characteristic of hereditary connective tissue disease; however, she did not undergo comprehensive evaluation. The patient underwent cesarean delivery followed by total hysterectomy to prevent uncontrollable obstetric bleeding. She subsequently underwent total arch replacement and the Bentall procedure. The patient's postoperative course was unremarkable, and neither the mother nor the newborn showed any complications. Genetic testing revealed Loeys-dietz syndrome in the mother.