Introduction. The incidence of aortic dissection ranges 3–5 cases per 100,000 person-years. In women under the age of 40 who acquire aortic dissection, almost half of the cases happen during pregnancy. Case outline. We report a case of a 22-year-old pregnant woman in the 16th gestational week with a history of arterial hypertension and known aortic dilatation, who was admitted with aortic dissection. She underwent the Bentall procedure, in which her aortic valve, ascending aorta, and proximal part of the aortic arch were replaced using valved composite graft. On the next day, the evacuation abortion was made, since the fetus didn’t survive the operation. Six years later, the patient stopped taking anticoagulation therapy and was admitted for prosthetic valve thrombosis, which was successfully treated with intravenous heparin. One year later, the patient died of a myocardial infarction due to coronary thromboembolism, confirmed on autopsy. Conclusion. Preconceptional counseling in women with a known aortic disease is of the utmost importance. Aortic dissection in pregnant women is an acute life-threatening condition for both mother and fetus that should be managed by a multidisciplinary team. After a mechanical heart valve implantation, lifetime oral anticoagulants are mandatory.