Waldenstrom macroglobulinemia is a rare cancer and is characterized by high level of immunoglobulin M (IgM) in the serum that causes increased serum viscosity and presence of a lymphoplasmacytic infiltrate in the bone marrow. Waldenstrom macroglobulinemia starts in B cells or B lymphocytes and leads to form large amount of IgM. Treatment options for Waldenstrom macroglobulinemia vary according to the symptoms, although standard treatments are available. In the last few years, medical science has made progress and number of new treatments for Waldenstrom macroglobulinemia have been introduced. However, very few studies have been done to compare the best treatment. Hence, there is no single treatment available that works for all patients of Waldenstrom macroglobulinemia. Symptomatic Waldenstrom macroglobulinemia patients are kept under observation and do not receive any treatment until symptoms show up. Waldenstrom macroglobulinemia patients showing symptoms such as anemia, hyperviscosity, cold-agglutinin disease, amyloidosis, and peripheral neuropathy are considered for the treatment. Patients that show symptomatic hyperviscosity receive plasmapheresis along with rituximab treatment. In most of the cases of Waldenstrom macroglobulinemia, combination therapy with chemo and immunotherapy is recommended. Patients with Waldenstrom macroglobulinemia may require stem cell transplantation in the later stage. Hence, standard chemotherapy agents such as chlorambucil, bendamustine, cladribine, or fludarabine are not prescribed as a first line therapy as they may affect the stem cells.