Purpose: Marfan syndrome (MFS) is an autosomal dominant genetic disorder of the connective tissue associated with progressive dilation of the aorta and a potential risk for aortic dissection. Objective of this study was to review the successful management of one high-risk pregnancy to term complicated by MFS. Material and Methods: Authors consulted the most important scientific databases investigating the influence MFS on pregnancy, analyzing fetal and maternal complications, gestational age at the time of delivery, labor, the postpartal fetomaternal complications, and neonatal and maternal outcome. Results: Obstetric complications associated with MFS may also include preterm delivery, preterm prelabor rupture of the membranes, cervical incompetence, and postpartum hemorrhage. In pregnancies complicated by MFS, the most common fetal and neonatal complications are preterm birth, small for gestational age (SGA), respiratory distress syndrome (RDS), intraventricular hemorrhage (IVH), fetal demise, perinatal mortality, and neonatal mortality (up to one month of life). Conclusion: The control of most high risk pregnancies, as this one complicated by MFS, must be multidisciplinary. The present authors’ attempt was to review the important aspects of the evaluation and management of a successful outcome of a pregnancy complicated by MFS.