Objective To study the clinical characteristics, therapy strategies and the outcomes of female patients with acute aortic dissection during late pregnancy and puerperal period. Methods We retrospectively analyzed the clinical data of 7 patients with acute aortic dissection during late pregnancy and puerperal period in Shanghai Changhai Hospital between August 2012 and June 2017. Five of the 7 patients were late stage pregnancy, 2 were puerperal period (1 at the postpartum night, 1 in 18 days after delivery). There were 6 patients of Stanford type A aortic dissection (85.7%), and 1 patient of type B aortic dissection (14.3%). The age of the patients ranged from 26 to 34 (30.8±3.1) years. Cardiac ultrasonography of patients with type A showed that the maximum diameter of the ascending aortas was 4.2–5.7 (4.7±0.6) cm, of which 2 patients were aneurysm of aortic sinus, 3 patients were with Marfan syndrome. Bentall procedure was conducted in 1 patient, Bentall+Sun’s surgery in 2 patients, ascending aorta replacement+Sun’s+coronary artery bypass grafting surgery in 1 patient, aortic root remodeling+ascending aorta replacement+Sun’s surgery in 2 patients. One patient with Stanford type B acute aortic dissection was performed with thoracic endovascular aortic repair (TEVAR) after cesarean section. Results Aortic blocking time ranged from 51 to 129 (85.5±22.9) min. Cardiopulmonary bypass time was 75–196 (159.0±44.0) min. Moderate hypothermic circulation arrest with selective cerebral perfusion time was 20–30 (23.8±3.5) min. All maternal and fetuses survived. The infant whose mother received aortic repair in early stage and then received cesarean section was diagnosed with cerebral palsy. Maternal and fetuses were followed up for 9 months to 4 years. During the follow up period, all the fetuses grew well except the cerebral palsy one, and all maternal recovered well. The patient who received aortic repair in the early stage, had a sigmoid rupture during cesarean section and was treated with sigmoid colostomy. Another patient with Stanford type A dissection was diagnosed as left renal vein entrapment syndrome after 2 years. Conclusion Type A aortic dissection is more common in late pregnancy and puerperal patients. And Marfan syndrome is a high-risk factor for acute aortic dissection in pregnancy women. Early and appropriate surgical treatment strategy based on the type of aortic dissection and gestational age are the key points to achieve good outcomes both for maternal and fetus.