Pax6 is a highly conserved transcription factor essential for the development of the eyes in vertebrate and invertebrate species. It is also required for normal development of many regions of the central nervous system, including the mammalian forebrain, hindbrain and spinal cord. In the forebrain, it is expressed in a gradient in the dorsal telencephalon, where it is required for the expression of genes that confer dorsal characteristics and where it might play a role in regionalization of the cerebral cortex. It is expressed in the diencephalon, where it is essential for the specification of its derivatives. While the ancestral function of Pax6 may have been to specify a structure sensitive to light, it has been co-opted into the regulation of a broader range of processes in development of the vertebrate nervous system.