Abstract: Marfan syndrome (MFS) is an autosomal dominant connective tissue disorder with multisystemic manifestations, which typically involve the cardiovascular, ocular and skeletal systems. Aortic aneurysm rupture and aortic dissection are the main causes of mortality in patients with MFS. Recent studies reveal that upregulated transforming growth factor β (TGF-β) and its noncanonical signaling are related to the development of vascular diseases and that miR-29b plays a key role in early development of aortic diseases in MFS.