Genetic variants in genes including TGFBR1, TGFBR2, TGFB2, TGFB3, SMAD2, and SMAD3 result in Loeys–Dietz syndrome and are reported to cause Marfan-like phenotypes and variants in FBN2 and COL3A1 result in congenital contractural arachnodactyly and Ehlers–Danlos syndrome type IV [11,36]. Here, FBN2 is linked to congenital contractural arachnodactyly.