Extracellular vesicles (EVs) are heterogeneous cell-derived membranous vesicles which carry a large diversity of molecules such as proteins and RNA species. They are now considered to be a general mode of intercellular communication by direct transfer of biomolecules. Emerging evidence demonstrates that EVs are involved in multiple pathological processes of brain diseases including neurodegenerative disorders. In this review, we investigate the current knowledge about EV biology. We also provide an overview of the roles of EVs in related brain diseases, particularly in neurodegenerative disorders. Finally, we discuss their potential applications as novel biomarkers as well as the developments of EV-based therapies.