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Background Marfan syndrome is a genetic connective tissue disorder that commonly affects the cardiovascular, skeletal, and ocular system. The increased risk of developing thoracic aortic aneurysms that can lead to aortic dissection and rupture is the main source of mortality in these patients. Pregnancy-induced changes can further increase the risk for aortic complications, especially in patients with an aortic root diameter > 45 mm. Case summary The case of a 26-year-old female with Marfan syndrome who was lost to follow-up for five years and presented to our department being pregnant at 21 weeks is presented. Echocardiography and cardiovascular magnetic resonance (CMR) showed an aortic root diameter of 55 mm and a large aneurysm of an aberrant right subclavian artery. Following multidisciplinary team discussion, valve-sparing aortic root and ascending aortic replacement was performed at 22 weeks of gestation without any complications. During the remaining pregnancy, the patient had frequent clinical and CMR follow-up investigations showing a mild increased size of the subclavian aneurysm. Uncomplicated caesarean delivery was performed at 35 weeks of gestation, and the subclavian artery aneurysm was successfully treated by interventional embolization. Discussion Although cardiovascular surgery in our patient during pregnancy was uncomplicated, the case illustrates that pre-pregnancy counselling in Marfan patients is recommended to reduce the risk for mother and child. [ABSTRACT FROM AUTHOR]