Non-Hodgkin lymphomas (NHLs) include any kind of lymphoma except Hodgkin’s lymphoma. Mantle cell lymphoma (MCL) is a B-cell NHL and accounts for about 6% of all NHL cases. Its epidemiologic and clinical features, as well as biomarkers, can differ from those of other NHL subtypes. This article first provides a very brief description of MCL’s epidemiology and clinical features. For etiology and prognosis separately, we review clinical, environmental, and molecular risk factors that have been suggested in the literature. Among a large number of potential risk factors, only a few have been independently validated, and their clinical utilization has been limited. More data need to be accumulated and effectively analyzed before clinically useful risk factors can be identified and used for prevention, diagnosis, prediction of prognosis path, and treatment selection.