It is important to recognize chronic inflammatory demyelinating polyneuropathy, which has features in common with the acute variant of inflammatory demyelinating polyneuropathy (Guillain–Barré syndrome), since corticosteroids, intravenous immune globulin, plasma exchange, and immunosuppressive agents may ameliorate its course. This review summarizes present knowledge about clinical presentation, diagnostic criteria, and diagnostic procedures involved in assessing this condition, as well as its current management.