Background <p>Acute type A aortic dissection (ATAAD) is very rare during pregnancy, it is a devastating condition, and there is no established management guideline. This study shares our center’s observations and management experience to provide a practical reference for developing individualized clinical strategies.</p> Methods <p>A total of 12 pregnant patients with acute type A aortic dissection, confirmed by CTA and managed surgically, were included from Union Hospital of Fujian Medical University between January 2014 and September 2024.</p> Results <p>A total of 12 patients were included, with a mean age of 30.83 years and a gestational age at admission ranging from 11 weeks and 4 days to 40 weeks and 1&#xa0;day. Comorbidities included Marfan syndrome (<i>n</i> = 4), gestational hypertension (<i>n</i> = 1), and diabetes mellitus (<i>n</i> = 2). Surgically, 7 patients underwent a Bentall procedure, 4 underwent an isolated Sun’s procedure, and 1 received a combined Sun’s procedure and coronary artery bypass grafting (CABG). The in-hospital mortality rate was 16.7% (2/12). There were four fetal deaths, one due to preexisting malformation and three following elective termination due to early gestational age. At the 1-year follow-up, all surviving patients and their newborns were living normally without significant complications.</p> Conclusion <p>This single-center study demonstrates that for pregnant patients with ATAAD, timely surgical repair, when managed by a multidisciplinary team, is a feasible strategy and is associated with favorable maternal and fetal outcomes.</p>