The clinical use of cancer immunotherapy with immune checkpoint inhibitors has transformed the management of cancer and added another effective treatment option for different types of malignancies. The blockade of immune checkpoint pathways triggers an enhanced immune response leading to cancer regression but may also lead to autoimmune toxicities or immune-related adverse events, which may involve skin, endocrine, respiratory, gastrointestinal or neurologic manifestations. Clinically relevant neurologic complications involving the central and/or peripheral nervous system affect up to 1% of patients treated with immune checkpoint inhibitors and may be associated with significant morbidity and mortality. Common neurologic complications include aseptic meningitis and encephalitis, hypophysitis, myasthenia, myositis and neuropathies. Neurologic immune-related adverse events after immune checkpoint inhibition should be distinguished from cancer progression or other complications of cancer therapy (e.g. infections). The treatment of neurologic complications may include holding or withdrawing cancer immunotherapy, anti-inflammatory and immunosuppressive therapies with corticosteroids and steroid-sparing agents, immunomodulation with intravenous immune globulin or plasmapheresis and symptomatic treatment (e.g. antiepileptic medications, pain medications).