Mantle cell lymphoma (MCL) is a unique lymphoma subtype comprising 6% to 8% of new lymphoma diagnoses. It is generally considered to be incurable with standard lymphoma therapies. The median overall survival (OS) is often reported to be 3 to 4 years, but more recent data suggest that the median OS may be longer than 5 years. There is considerable heterogeneity in MCL, with some patients succumbing to their disease in less than a year and others surviving for more than 10 years. The biology of MCL is reasonably well understood, and targeted therapies based on this knowledge are in development. Clinical trials incorporating new agents into standard therapies are under way. The optimal frontline treatment strategy is not defined and is a source of controversy. Intensive therapies administered to younger patients appear to produce better-quality remission than standard treatments, and one randomized clinical trial demonstrates improved OS with an intensive approach. Additional randomized clinical trials defining standard approaches are needed.