Here, we report global and pleiotropic PCP defects in Prickle1 null and hypomorphic mutants that closely resemble Wnt5a, Ror or Ryk mutant mice and recapitulate features of human Robinow syndrome (RS; RRS, MIM#268310, http://omim.org/entry/268310; DRS, MIM#180700, http://omim.org/entry/180700), prompting us to explore the link between Prickle1 and the Wnt pathway. Here, PRICKLE1 is linked to X-linked retinoschisis.