Paraneoplastic causes are a rare but important diagnostic consideration when evaluating myelopathy because neurologic symptoms may herald a diagnosis of cancer. Spinal cord MRI findings of longitudinally extensive, symmetric, tract-specific T2-signal changes occasionally with gadolinium enhancement are characteristic. Detection of neural-specific autoantibodies assists in confirming the diagnosis and guides the cancer search. Initial management involves detection and treatment of the underlying cancer. Combinations of immunotherapies are typically recommended but evidence-based therapeutic guidelines are lacking and morbidity remains high. Autoimmune myelopathies may also occur in association with neural-specific autoantibodies without an underlying cancer and in association with systemic autoimmune disorders.