Neurodegenerative diseases are chronic and progressive disorders which are not effectively treated through adopting conventional therapies. For this unmet medical need, alternative therapeutic methods including gene-based therapies are emphasized. MicroRNAs (miRNAs) are small non-coding RNAs which can regulate gene expression at the post-transcriptional level. In recent years, dysregulated miRNAs have been indicated to be implicated in the occurrence and development of neurodegenerative diseases. They are investigated as candidates for diagnostic and prognostic biomarkers, as well as therapeutic targets. The miR-200 family consists of miR-200a, -200b, -200c, -141, and -429. Numerous studies have found that miR-200 family members are associated with the pathogenesis of neurodegenerative diseases. It is reported that miR-200 family members are aberrantly expressed in several neurodegenerative diseases, participating in various cellular processes including beta-amyloid (Aβ) secretion, alpha-synuclein aggregation and DNA repair, etc. In the present review, we summarize the recent progress in the roles of miR-200 family in neurodegenerative diseases.