The pathological features of neurodegenerative diseases mainly include the accumulation and deposition of abnormally misfolded proteins or peptides. Autophagy can clear intracellular aggregated proteins and dysfunctional organelles, making it an important target for delaying the progression of neurodegenerative diseases. Impairments in the production or maturation process of autophagosomes can promote the development and progression of neurodegenerative diseases. A thorough understanding of the signal transduction pathways regulating autophagy is crucial for devising treatment strategies for related diseases. This paper mainly introduces the important role of autophagy in neurodegenerative diseases and its associated molecular mechanisms.