Paraneoplastic myelopathies are uncommon, but maintenance of a high index of clinical suspicion is critical, as the neurological syndrome often predates discovery of an underlying malignancy and can facilitate early detection and potentially improve prognosis. Most paraneoplastic myelopathies are associated with additional neurological symptomatology, such as encephalitis, peripheral neuropathy, or cerebellar ataxia, due to the specific, respective auto-antibodies involved. Anti-Hu antibody, identified in our patient, appears to be the most common antibody, followed by amphiphysin, and collapsing response mediator protein 5, among others. The therapeutic approach to patients with paraneoplastic myelopathies focuses primarily on treatment of the underlying cancer and, thereafter, subsequent trials of immunotherapies, which have demonstrated variable response.