Paraproteinemic neuropathies comprise a diverse group of disorders that includes monoclonal gammopathy of undetermined significance, primary amyloidosis, multiple myeloma, cryoglobulinemia, POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein and skin changes) syndrome, and Waldenstrom macroglobulinemia. Various factors including hepatitis C virus, vascular endothelial growth factor, and an array of cytokines are implicated in the pathogenesis of these conditions. More recently, a variety of novel antibody specificities, and vasculitis, have also been described as contributory factors in the development of these neuropathies. Therapeutic approaches for paraproteinemic neuropathies have included administration of cytotoxic agents, steroids, interferon-alpha, intravenous immunoglobulin, radiation, bone marrow transplantation, and more recently, drugs such as rituximab and bevacizumab. In this article, we review some of the well-known features of these diseases, and highlight some of the more recent findings from the vast literature for these diseases.