Aortic dissection during pregnancy is a very rare event in the general population but can be fatal to both the mother and the fetus. A rate of dissection as high as 10% was observed in pregnant patients affected by Marfan syndrome. Facing this kind of disease can represent a challenge for the involved physicians because of its rarity. Here we present the case of an aortic dissection in a pregnant woman with Marfan syndrome who previously underwent an open heart surgery for a mitral prolapse. The diagnosis and the treatment of this case, given the mid-term gestational age combined with an increased surgical risk due to the reintervention, required a particular effort by our team. A multidisciplinary approach to the management of this patient was the key to achieve a favorable outcome both for the mother and for the baby.