Loeys-Dietz syndrome (LDS), an autosomal dominant genetic disorder, is linked withvariants in a signaling pathway termed transforming growth factor-β(TGF-β) involving variants in TGF-β receptors, TGFBR1 & TGFBR2,TGF-β ligands, TGFB2 & TGFB3, and SMAD2 and SMAD3[1,2,3,4]. The gene discussed is TGFB1; the disease is Loeys-Dietz syndrome.