Waldenström macroglobulinemia (WM) is a lymphoid neoplasm characterised by a monoclonal lymphoplasmacytic expansion accompanied by a serum monoclonal immunoglobulin M (IgM). In some patients, the monoclonal protein will lead to a characteristic hyperviscosity syndrome. Although indolent, WM is incurable and most patients eventually succumb to disease progression. Thus, we need to better understand the natural history and biology of the disease. Recent work has shown that half of patients with WM harbour deletions in the long arm of chromosome 6. Increasing evidence suggests the disease is a defined pathological entity and not purely a clinical syndrome. Current therapeutic modalities include alkylator agents, purine nucleoside analogues, and rituximab. The optimum initial therapeutic strategy is not yet defined, and current clinical trials are addressing the role of combination therapy. In this review, we summarise the current understanding of the pathogenesis, clinical and laboratory features, prognostic factors, and therapeutic options for patients with WM. We also discuss current knowledge of WM and available therapies.