In the patients with neoplastic disease localized in the organs other than nervous system different neurological symptoms can be observed. Some of them constitute paraneoplastic neurological syndromes (PNS), which are distant manifestations of neoplasms. PNS are thought to be caused by autoimmune processes triggered by the cancer and directed against antigens common to both the cancer and nervous system. They can be associated with the presence of onconeural antibodies. This article provides an overview on the clinical presentation, diagnosis, pathophysiology and treatment of PNS.