Type A acute aortic dissection (TAAD) during pregnancy is a life-threatening event for both the mother and unborn baby. Pregnancy has been recognised as an independent risk factor for TAAD, postulated to be due to physiological changes that cause hyperdynamic circulation. Presentation can be atypical in many cases and further concern from clinicians of fetal radiation exposure can result in missed or delayed diagnoses. Investigation via quickest form of imaging, whether CT, MRI or transoesophageal echocardiography, should be carried out promptly due to the high risk of mortality. Surgical management of TAAD in pregnancy revolves primarily around the decision to deliver the foetus concomitantly or to perform aortic repair with the foetus in utero. This review will summarise the difficulties faced when managing TAAD in pregnancy, and important questions for future research.