Mantle cell lymphoma is a rare yet well defined subtype of B-cell non-Hodgkin's lymphoma. The correct histological diagnosis of this lymphoma subtype is of the utmost importance; however, it is also a very difficult diagnosis. Clinical management is often complex, and despite the successful introduction of monoclonal antibodies and dose-intensified regimens, including autologous and allogeneic stem cell transplantation strategies, the prognosis remains particularly poor. Recently gained insights into the underlying biology and pathogenesis have unravelled numerous promising molecular targeting strategies; however, their introduction into clinical practice and current treatment algorithms remains a challenge. This article addresses these issues providing relevant information for current state-of-the-art management of patients with mantle cell lymphoma and giving a perspective of upcoming treatment strategies.