Paraneoplastic disorders of the nervous system (PNDs) are rare and unique disorders, where a specific pattern of neural damage occurs as a side effect of the interaction between the neoplasm and the host immune response. Clinical recognition of PNDs may be challenging but can lead to early detection of an occult neoplasm. Their study may lead to a better understanding of nervous system autoimmunity and even to devising novel immunotherapies against certain tumor types. Familiarity with the clinical syndromes, neuroradiological findings, autoantibodies, and tissue responses associated with PND may help arrive at a correct diagnosis in most cases.