Neurological paraneoplastic syndromes (NPS) affect only 0.01% of cancer patients, chiefly those affected by lung, breast, ovarian and stomach cancer. They frequently cause major disability and produce limitations in patients' daily activities; the character of the disease is irreversible. Clinical suspicion is fundamental for an early diagnosis and it must be backed up by the specification of certain antibodies both present in blood and in cerebrospinal fluid (CSF). Conventional treatments are very inefficient at the time of treating these disorders; at present, the administration of immunoglobulins, immunosuppressors, chemotherapy agents and corticoids are under study, but so far results are not promising. The aim of this review is to analyze the variety of NPS and describe the findings concerning autoimmunity and treatments used at present.