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Immune-checkpoint inhibitors (ICIs) constitute a novel class of agents recently approved to treat a number of human malignancies. Due to their immunomodulatory mechanism of action, ICIs can generate a wide range of immune-related adverse events (irAEs) of which neurological toxicities are of special interest because of their potential severity. The objective of this review is to examine the recent literature describing neurological irAEs and discuss their optimal management.As opposed to irAEs involving other organs, neurological complications of ICIs are uncommon. These complications encompass various toxicities of the central and peripheral nervous systems, including myositis, myasthenia gravis, demyelinating polyradiculoneuropathy, meningitis and encephalitis. Neurologic irAEs are often responsive to corticosteroids and other immune-modulating treatments (e.g. plasmapheresis, intravenous immunoglobulin), which have been used in patients presenting with severe neurologic irAEs or irAEs unresponsive to corticosteroids. Data from literature indicate that early treatment is critical for reducing the morbidity associated with neurologic irAEs.ICI-associated irAEs constitute a new group of neurologic complications of systemic anticancer therapies. Although potentially severe, these rare neurologic toxicities are often responsive to immune-modulating therapies. Early recognition and treatment is crucial for timely improvement of functional outcome and requires a multidisciplinary approach.