POEMS syndrome with its classical five findings (Polyneuropathy, Organomegaly, Endocrinopathy, M protein, and Skin changes) is a rare multisystem disease. Proinflammatory and proangiogenic cytokines play important roles in its pathogenesis. Treatment options are still debated.We present a 65-year-old man with POEMS syndrome who was successfully treated with bortezomib.After seven cycles of this protocol, serum M protein level declined to normal range, and near-to-complete remission was achieved. His symptoms of polyneuropathy improved dramatically.Bortezomib may be an effective and safe therapeutic option for patients with POEMS syndrome.