Treatment with checkpoint inhibitors has revolutionised the treatment of many different cancers with a significant improvement in cancer survival. However, a different type of adverse effects is seen compared to standard chemotherapy called immune-related adverse effects (irAE). In this review, we give recommendations on how to diagnose and treat the most common and severe neurologic irAE. We advise clinicians to be cautious to classify neurologic irAE as well-known nosologic entities, since their presentation, cause and treatment often is different.