Planar cell polarity (PCP) refers to coordinated polarization of cells in the plane of a cell sheet. In Drosophila, the stereotypical arrangement of the eight photoreceptor cells in each of the ommatidia of the fly compound eye and the uniform orientation of the hairs in all the wing cells are two representative forms of PCP. Using these powerful Drosophila model systems, a set of genes was identified to constitute the invertebrate PCP signaling pathway. In vertebrates, the inner ear sensory organs display distinctive forms of PCP. In particular, the auditory sensory organ in the cochlea, adorned with precisely patterned sensory hair cell arrays and uniformly oriented hair bundles, has served as an excellent model system to complement other vertebrate PCP models and has illustrated a genetic pathway that consists of genes conserved from the Drosophila model as well as genes uniquely required for vertebrate PCP regulation. This review will focus on the mouse models that have made valuable contributions to our current understanding of PCP signaling in the vertebrates.