Autoimmune disorders are increasingly recognized causes of several neurological disorders leading to significant clinical disability. This article reviews recent developments in our understanding on the pathophysiology, clinical presentations, and diagnoses of selected immune-mediated neurological disorders. It also provides a brief summary of current theories on autoimmunity and the role that certain environmental factors play in the development of immune-mediated neurological disorders. Recently recognized biomarkers might play a pathogenetic role or simply serve as a diagnostic tool.