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Type I monoclonal cryoglobulinemia is usually associated with lymphoproliferative disorders, such as monoclonal gammopathy of undetermined significance (MGUS), myeloma, chronic lymphocytic leukemia (CLL) and lymphoplasmocytic lymphoma (LPL). Clinical symptoms related to Type I cryoglobulin (CG) isotype often include skin, neurological and renal manifestations.A 42-year-old woman who initially presented urticaria, palpable purpura in both her upper extremities and legs, eosinophilia and Raynaud phenomenon. Skin biopsy revealed eosinophil infiltration. Monoclonal immunoglobulin (Ig) G-κprotein was detected and CG was also positive.The patient was finally diagnosed as MGUS related Type I CG.Cyclophosphamide-dexamethasone-thalidomide (CDT) therapy was initiated.The treatment relieved the skin symptoms efficiently.To our knowledge, this is a rare case of Type I cryoglobulinemic vasulitis with eosinophilia complicated by MGUS, and the effective treatment of cyclophosphamide combined with thalidomide and prednisone may provide a new therapeutic option for cryoglobulinemic vasulitis.