Marfan syndrome(MFS)is an autosomal dominant heritable disorder of connective tissue.Recent studies showed that regulation of transforming growth factor beta(TGF-β)signaling may play an crucial role in MFS.What'more,Transforming Growth Factor Beta Receptor Type Ⅱ(TGFBR2)and Transforming Growth Factor Beta Receptor TypeⅠ(TGFBR1)mutations were identified in part of patients with MFS.This article reviewed the progress of the research on TGF-β and its receptor with MFS.