A dominant form of RS is caused by mutations in Wnt/PCP components DVL1, DVL3, and WNT-5A, it is therefore believed that the developmental defects seen in Robinow syndrome are caused by a deregulation of Wnt-5a/Ror2/PCP signaling (Stricker et al., 2017). Here, WNT5A is linked to Robinow syndrome.