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. This evidence concerns the gene CD40LG and MALT lymphoma.