Pregnancy with aortic dissection is dangerous and easy to be misdiagnosed and to escape diagnosis. The risk of adverse outcomes in mother and child is extremely high, which is closely related to the classification of the dissection. The cause of aortic dissection in pregnancy is mainly related to primary hypertension, congenital and hereditary vascular disease, pregnancy is the risk factor. Chest pain is the main clinical manifestation, and the diagnosis mainly depends on imaging examination. Treatment includes supportive therapy, drug therapy, traditional surgical treatment, thoracic endovascular aneurysm repair, etc. The treatment, timing of delivery and mode of delivery are determined according to the extent and type of aortic dissection, gestational age, patient and family wishes, and medical conditions. Strengthening pre-pregnancy and pregnancy care, multidisciplinary assessment and treatment are the keys to reduce poor outcome of mother and fetus.