Type A aortic dissection (AAD) is one of the most challenging emergencies in cardiac surgery with an early high mortality rate if untreated. It is a very rare complication during pregnancy, favored by systemic hypertension, hypervolemia and hormonal changes weakening the aortic wall. A 22-weeks (w) pregnant woman experienced sudden left lower limb paresthesia and isolated motor aphasia. Clinical history revealed prior gestational hypertension and familiarity for AAD. At our Emergency Dept echocardiography and CT revealed AAD extending from aortic root to all the supra-aortic trunks and both the femoral arteries. A moderate aortic valve regurgitation was observed. Uterine arteries were not involved with a good placental perfusion. Multidisciplinary evaluation led to emergency cardiac surgery aimed to preserve mother’s life first, considering prohibitive the gestational age for a preoperative emergency c-section, and judging the risk of hemorrhage from pregnancy interruption as unacceptable. Due to the status of the peripheral vessels, cardiopulmonary bypass (CPB) was established through bicaval and left ventricular apex cannulation. Ascending aorta and proximal arch were replaced and aortic valve commissures were resuspended. Cerebral and splanchnic protection were guaranteed by moderate systemic hypothermia during the circulatory arrest, antegrade cerebral perfusion and NIRS monitoring. Myocardial protection consisted of retrograde cold blood cardioplegia repeated every 20 minutes, alternated to hematic retrograde cold perfusion and left ventricular venting. Specific strategies were employed to ensure also fetal preservation during the procedure: strict control of blood potassium level to avoid fetal cardiac arrest, 22°C systemic hypothermia, short period of circulatory arrest. Particularly, we maintained systemic CPB flow at 130% of the target flow according to mother’s body size, in order to guarantee adequate placental perfusion. Obstetric ultrasound confirmed fetal vitality both at the start and completion of surgery and no other monitoring was indicated due to the small gestational age. The subsequent course of the pregnancy was uneventful, resulting in safe delivery via c-section at 35w. This case demonstrates that even in high-risk emergency cardiac surgery, the implementation of tailored surgical and perfusion strategies can successfully preserve the lives of both the mother and the fetus.