Abstract: Mantle cell lymphoma (MCL) is a rare subtype of B-cell non-Hodgkin's lymphomas (NHLs). MCL comprises distinct subtypes with different pathological characteristics and clinical features. However, MCL remains incurable by intensified first-line regimens con-taining cytarabine and involving consolidation with high- dose therapy and autologous stem cell transplantation. Recently discovered somatic mutations and aberrant intracellular signaling pathways have been demonstrated to play an essential role in the pathogenesis of MCL. The related novel therapeutics, such as Btk inhibitors, PI3K inhibitors, and immune modulators, have exhibited promising ther -apeutic effects on untreated or even relapsed/refractory MCL. The development of an efficient combination therapy is urgently needed to improve the survival of MCL patients in the future.