Background <p>Acute type A aortic dissection during pregnancy is a rare but life-threatening condition, with an estimated incidence of approximately 4 cases per 1&#xa0;million women aged 15–45 years. Hormonal and hemodynamic changes during pregnancy are factors that may predispose to aortic dissection. These changes begin in the first and second trimesters but are most pronounced in the third trimester and the early postpartum period. Genetic syndromes, such as Marfan syndrome, significantly increase the risk of aortic dissection.</p> Case presentation <p>A 41-year-old pregnant woman at 31 weeks of gestation with genetically confirmed Marfan syndrome presented with acute, severe, sharp retrosternal chest pain lasting four hours, radiating to the neck, accompanied by weakness and dizziness. Transthoracic echocardiography revealed dilation of the ascending aorta without significant aortic valve regurgitation. Computed tomography angiography confirmed the diagnosis of Stanford type A aortic dissection. Following a multidisciplinary team decision, an initial cesarean section with subsequent uterine artery embolization was performed, followed by emergency ascending aortic replacement. The patient and the newborn were successfully discharged on postoperative day 20.</p> Conclusions <p>Although aortic dissection during pregnancy is rare, clinicians must maintain a high suspicion index for pregnant women presenting with severe chest pain. Careful monitoring of patients with suspected or confirmed connective tissue disorders is essential. This case demonstrates that acute type A aortic dissection may occur in pregnant patients with Marfan syndrome despite only moderate aortic root enlargement and highlights the value of rapid multidisciplinary management, including coordinated obstetric, endovascular, and cardiac surgical intervention.</p>