Cdx1, Cdx2 and Cdx4 are members of the caudal-type homeobox family of genes, which are homologs of the Drosophila “caudal” gene required for anterior-posterior regional identity. The proteins encoded by these genes are transcription factors which play an important role in development by regulating the expression of Hox genes. Hox genes play a fundamental role in the development of the vertebrate central nervous system, heart, axial skeleton, limbs, gut, urogenital tract and external genitalia. Cdx4 is a major positive regulator of the expression of all Hox family members. Due to its critical role as a regulator, Cdx4 is a direct target of the canonical Wnt pathway. The loss of Cdx4 can result in the development of an expanded hindbrain, while the overexpression of Cdx4 may cause the hindbrain to lose its distinct segmental features and resemble the spinal cord.