Through a literature search, we retrieved 4 cases of MZL lymphoma with first peripheral neuropathy, the serum M protein of these 4 cases was monoclonal IgM type.[8–10] We presented the first report of the coexistence of primary mediastinal MALT lymphoma and MM like POEMS syndrome, which first presented with peripheral neuritis. The gene discussed is CD40LG; the disease is peripheral neuropathy.