Ciliary neurotrophic factor (CNTF), originally identified for its ability to promote survival of neurons of the ciliary ganglion, is now known to have additional survival and differentiative actions on cells of the nervous system. CNTF is, however, unrelated in structure to the nerve growth factor family of neurotrophic factors. Instead, CNTF is distantly related to, and in fact shares receptor components with, a number of hemopoietic cytokines. This review focuses on the biological actions of CNTF, the shared and unique features of the CNTF receptor complex and signaling pathways, and the distribution of CNTF and its receptor during development, in the adult and in response to injury.