While SMAD6 variants, including the frameshift here reported, have been proposed to contribute to craniosynostosis, in association with a common polymorphism within the BMP2 locus (rs1884302) [20], this association has not been confirmed in additional craniosynostosis cohorts nor in cardiovascular/RUS patients [15,18,19,21,22]. This evidence concerns the gene BMP2 and craniosynostosis.