The hedgehog (Hh) pathway is a highly conserved signalling cascade involved in many developmental processes, including a key role in morphogenesis of many tissues including the limb bud, lung, gut, hair follicle and the neural tube. Hh role in adult tissue is less well-established, however, it is known that the pathway becomes activated and reutilised in situations of repair and regeneration. In the nervous system, tissue repair appears impeded in that mature neurons undergo their final cell divisions early in life and central axons do not easily regenerate. The Hh pathway has been shown to be activated in response to nerve damage, leading to the hypothesis that enhancing Hh pathway activation in damaged nerve tissue, inducing the repair process, could offer a potentially new approach to treating neurodegenerative diseases and dysfunction, including spinal cord injury.