Objective To review research progress of the relation between glial cell line-derived neurotropic factor (GDNF) and motoneuron development and motoneuron disease. Methods The recent articles on GDNF and motonerons were extensively reviewed. The molecular structure, the mode of action and the route of administration of GDNF were investigated. Results GDNF plays extensive roles in the development anddisease of motoneuron. GDNF might regulate the development of the motonerons of the spinal cord to some extent and also save the injured motoneurons. Conclusion GDNF has a potential clinical value and inestimable futurein the treatment of motoneuron diseases.