Among documented cases of POEMS syndrome, all of them have included peripheral nerve damage.[5] However, Ryuji Morizane's team reported a case of atypical POEMS syndrome without polyneuropathy in 2008.[6] Here, we describe the case of a patient who presented with inflammatory myopathy and several typical characteristics of POEMS syndrome, including skin manifestations, lymphadenopathies, pedal edema, IgA-λ restricted paraproteinemia, and elevation of VEGF and other features, but peripheral nerve conduction tests were normal. The gene discussed is VEGFA; the disease is Paraproteinemia.