Extracellular vesicles (EVs) have emerged as pivotal mediators in neurological diseases, showcasing multifaceted potential roles ranging from pathogenesis to diagnosis. These nano-sized membranous structures, released by various cell types including neurons, astrocytes, and microglia, encapsulate a diverse cargo of proteins, lipids, RNA species, and even DNA fragments. In neuropathology, EVs contribute significantly to intercellular communication within the central nervous system (CNS), influencing physiological or pathological cascades. Through the transfer of bioactive molecules, EVs modulate neuroinflammation, neuronal survival, synaptic plasticity, and the propagation of protein aggregates characteristic of neurodegenerative disorders. Moreover, their presence in biofluids such as cerebrospinal fluid (CSF), blood, and urine reflects the pathophysiological state of the CNS, offering a window into diagnosis, monitoring and treatment of neurological diseases. Recent advancements in EV isolation techniques, coupled with high-throughput omics technologies, have facilitated the profiling of EV cargo, enabling the identification of disease-specific biomarkers with high sensitivity and specificity. This review explores the intricate roles of EVs in neuropathology, highlighting their involvement in Alzheimer’s disease, Parkinson’s disease, Multiple Sclerosis, and other neurological disorders. Furthermore, it delves into the diagnostic potential of EVs, discussing current challenges and prospects in harnessing EV-derived biomarkers for precision medicine in neurology. Ultimately, understanding the biology of EVs in neurological contexts promises transformative insights into disease mechanisms and therapeutic strategies, paving the way for innovative diagnostic tools and targeted interventions in clinical practice.