This review focuses on recent developments in the treatment of inflammatory neuropathies arising from immune dysregulation, rather than from infectious causes. The dysimmune inflammatory neuropathies are diseases of the peripheral nerves that have varying etiologies and may respond to immunomodulatory therapies. They are characterized by inflammatory changes in the nerve with associated destruction of myelin and axons. The underlying immune mechanisms are better understood in some of these conditions than others. Correct diagnosis and treatment is important to prevent clinical progression. Randomized controlled trials of some treatments in the more common inflammatory neuropathies have clarified their effectiveness; however, there are still groups of patients who are resistant to currently available treatments and for whom little effective treatment is available. Newer, targeted biologics and larger controlled trials of existing and novel therapies in these conditions offer promise of improved morbidity and mortality in this group of diseases.