In recent years, immune checkpoint inhibitor (ICI) has achieved remarkable success in cancer therapy. However, the occurrence of immune-related adverse event (irAE) has increasingly attracted attention. Among them, neurologic immune-related adverse event (n-irAE) is relatively uncommon, but can cause severe outcomes. n-irAE can involve multiple regions of the nervous system, including the peripheral nerves, neuromuscular junction, and central nervous system (CNS), and lead to myositis, myasthenia gravis, encephalitis, myelitis, and other disorders. The diagnosis of n-irAE requires a combination of evaluating the time correlation between symptoms and ICI medication, neurological localization assessments (neuroimaging, cerebrospinal fluid analysis, and electrophysiological studies), and the exclusion of alternative etiologies such as metastatic disease or infection. n-irAE is graded and treated based on clinical severity, and the common therapeutic approaches include corticosteroids, intravenous immunoglobulin, and plasma exchange. Multidisciplinary collaboration and early intervention are crucial for improving patient outcomes. Future efforts should focus on optimizing risk prediction models to achieve individualized management of n-irAE.