Treatment of immune-mediated neuropathies first requires an accurate diagnosis. The diagnosis is often based on clinical, electrophysiological, and immunological features of the syndrome. The selection of appropriate therapies is then based on the spectrum of response of a syndrome to medications and an assessment of possible side effects. In neuropathies with associated serum immunoglobulin M autoantibodies, such as anti-myelin-associated glycoprotein and motor syndromes, the choices of therapy are often limited to cytotoxic agents and, in some cases, intravenous immunoglobulin. In neuropathies with immunoglobulin G antibodies in both serum and cerebrospinal fluid, such as sensory neuronopathies associated with anti-Hu antibodies, there is no well-documented response to any immunotherapy. The general principles regarding therapy of immune neuropathies will be discussed with a focus on diagnosis and treatment options of the demyelinating and immunoglobulin M antibody-associated neuropathies.