Lymphoplasmacytic lymphoma (LPL) is a rare low-grade lymphoma characterized by a mixture of small lymphocytes, lymphocytes with plasmacytoid features, and plasma cells, which do not fulfill the criteria for any of the other small B-cell lymphoid neoplasms [1–16]. It involves the bone marrow (BM) and occasionally the lymph node, spleen, and rarely blood. Monoclonal paraprotein (usually IgM) is often present, but is not required for the diagnosis of LPL. In most patients, adenopathy develops slowly over many years. Extramedullary sites may be involved as well, although most of these cases represent marginal zone B-cell lymphoma with plasmacytic differentiation. The most common extramedullary sites include the lymph nodes, soft tissue spleen, skin, lung, tonsil, gastrointestinal (GI) tract, and liver . Patients present with weakness and fatigue due to anemia usually have IgM serum paraprotein and may display hyperviscosity (up to 30%), neuropathy, cryoglobulinemia, or coagulopathy. Some patients have more lymphomatous manifestation with adenopathy or extranodal infiltrates.