A 34-year-old female with a gestational age of 38 weeks developed acute type A aortic dissection. Appearance of this patient was typical for Marfan's syndrome, and echocardiography revealed annulo-aortic ectasia with mild aortic regurgitation, but pericardial effusion was absent. As her hemodynamic condition was stable, an emergency Caesarean section was carried out first. After careful observation in the ICU for half a day, she successfully underwent aortic valve reimplantation and replacement of the ascending aorta under deep hypothermic circulatory arrest. Intraoperative heparin use minimally impacted uterine bleeding. Both the mother and the neonate were discharged home 16 days later. We believe a two-stage strategy should be adopted whenever possible.