Coexistence of neuropathy and paraproteinemia (monoclonal gammopathy) is a common and complex problem seen in clinical practice and requires the distinction of specific syndromes. The clinical courses of these neuropathies are typically chronic and progressive. A precise distinction of the type of haematologic disorder associated (benign or malignant), investigation of other organs manifestations, and assessment of specific markers are mandatory. These steps are important to initiate an appropriate therapy that may include chemotherapy and/or immunosuppressive treatment targeting the neuropathy and the haematological dysfunction.