POEMS syndrome (POEMS) is a rare multi-system disease which is typically associated with polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes. Because the peripheral neuropathy is frequently the overriding symptom and because the characteristics of the neuropathy are similar to that chronic inflammatory demyelinating polyneuropathy (CIDP), patients are frequently misdiagnosed with CIDP. Therapies that may be effective in patients with CIDP and not effective in patients with POEMS. We report herein a case of POEMS with progressive polyneuropathy resistant to high-dose intravenous immunoglobulin therapy, who presented with muscle weaknes, marked fatigue and paresthesia of the upper and lower extremities.