In Marfan syndrome, with dilatation of the aortic root secondary to an underlying connective tissue defect, pregnancy can cause hemodynamic stress leading to the development of an aortic aneurysm and even a fatal aortic dissection. In the presence of existing aortic root enlargement and a family history of aortic dissection, preventative elective surgery is suggested. Aortic root replacement with or without a valve-sparing procedure is superior to total aortic root replacement with prosthetic valve/tube graft. It provides excellent survival with low rates of aortic - valve related complications.