Patients with aortic dissection during pregnancy and postpartum period exhibit high mortality. At present, a complete overview of aortic dissection during pregnancy and postpartum period is lacking. This systematic review includes 80 reports published from 2000-2020, comprising a total study population of 103 patients with aortic dissection. It was suggested that Stanford Type A aortic dissection is more likely to occur in the third trimester, while Stanford Type B is more likely to occur within 12 weeks postpartum. The most common risk factor was connective tissue disease, with no other known risk factors. Mode of delivery has no significant effect on the type of postpartum aortic dissection. Reduced maternal and fetal mortality was observed when patients with Stanford Type A aortic dissection occurring after 28 gestational weeks underwent aortic replacement after cesarean section. Patients with Stanford Type B aortic dissection were treated mainly with medication and/or endovascular repair. Contemporary management of patients during pregnancy and within 12 weeks postpartum requires multidisciplinary cooperation and includes serial, non-invasive imaging, biomarker testing, and genetic risk profiling for aortopathy. Early diagnosis and accurate treatment are essential to reduce maternal and fetal mortality.