Waldenström s macroglobulinemia is an uncommon B-cell lymphoproliferative disorder characterized by bone marrow infiltration of luymphoplasmatic lymphoma, although sometimes there is a extramedullary lymphoma (nodal), with demonstration of an IgM monoclonal gammopathy. It must be distinguished from monoclonal gammopathy of undetermined significance and IgM- related disorders. The median survival of patients with Waldenström s macroglobulinemia averages 5 years. Asymptomatic cases should not be treated. Symtomatic cases could be treated with alkylating agents, nucleoside analogues (fludarabine or cladribine), and the monoclonal antibody rituximab. For the management of hyperviscosity and neuropathies plasmapheresis could be used successfully.