Stanford type A acute aortic dissection (AAD) is a very rare complication, with potentially lethal consequences in pregnancy. In fact, pregnancy has been regularly associated with the possibility of aortic. dissection in almost half of young women. Herein, we present the case of a 38-year-old woman in her 37^th week of pregnancy. The patient’s medical history was indicative of G4L2Ab1(4 gestaitions,2 lives, 1 abortion). She developed persistent chronic pain in the neck, chest, and back, without nausea or vomiting while waking in the morning. The computed tomography angiogram was indicative of AAD. The medical decision was to perform a combination of cesarean section under general anesthesia and median sternotomy for the open aortic valve. The term newborn showed an Apgar score of 9-10. The coronary arteries were preserved and the valve was repaired at commissural positions. Dacron supracoronary tube graft was attached to the aortic anastomotic site and subsequently to the aortic arch. The patient was discharged on day three after surgery with a good general condition. Moreover, the results indicated that she and one of her brothers suffered from Marfan syndrome.