Exosomes are nano-sized, lipid bilayer vesicles secreted by cells. They carry essential bioactive molecules, such as proteins, nucleic acids, and lipids, and are widely present in bodily fluids including blood and cerebrospinal fluid. Exosomes transfer bioactive molecules to target cells through various mechanisms, including endocytosis, ligand-receptor interactions, or direct membrane fusion, and play crucial roles in intercellular communication, including facilitating intercellular information exchange, maintaining nerve-cell function, participating in immune responses, and providing nutritional support. Exosomes significantly promote signal transmission and intercellular communication in the central nervous system and are involved in the pathogenesis and development of diseases by participating in the spread of pathological proteins, regulating neuroinflammation, and the deposition of pathological proteins. Therefore, exosomes play key roles in the occurrence and development of neurodegenerative diseases, and their contents, especially proteins and miRNAs, are specific for given pathological and physiological states and are relatively stable during extraction and analysis. Hence, exosomes are ideal tools for diagnosing diseases, staging their courses, and assisting prognosis. This article further explores exosomes derived from blood, saliva, urine, and cerebrospinal fluid as potential diagnostic biomarkers for neurodegenerative diseases. As natural drug-delivery systems, exosomes have the advantages of biocompatibility, ability to cross biological barriers, target specificity, stability, and containing natural therapeutic molecules, which can effectively improve the precision and efficacy of drug delivery and reduce side effects, making them an ideal carrier for delivering drugs to the central nervous system. Therefore, exosomes hold great potential in the diagnosis and treatment of central nervous system diseases. This article systematically reviews the latest advances in exosome research directed toward specific neurodegenerative diseases, focusing on their roles played in disease pathogenesis, progression, diagnosis, and treatment, with the aim of providing theoretical support and a reference for the early diagnosis and treatment of these diseases.