The most prevalent pathological features of many neurodegenerative diseases are the aggregation of misfolded proteins and the loss of certain neuronal populations. Autophgy, as major intracellular machinery for degrading aggregated proteins and damaged organelles, has been reported to be involved in the occurance of pathological changes in many neurodegenerative disorders, including Alzheimer’s disease, Parkinson’s disease, Huntington’s disease and amyotrophic lateral sclerosis. In this review, we summarized most recent research progress in this topic and provide a new perspective regarding autophagy regulation on the pathogenesis of neurodegenerative diseases. Finally, we further discussed the signaling molecules in autophagy-related pathways as therapeutic targets for the treatment of these diseases. This article is protected by copyright. All rights reserved.