Mantle cell lymphoma (MCL) is characterized by heterogeneous biology and varied clinical presentations. Historically, it has been associated with a poor prognosis when compared with other non-Hodgkin lymphomas. With a better understanding of the disease biology, molecular pathogenesis, and new treatments, the outcomes have been gradually improving. Identification of high-risk mutations has resulted in better prognostication and paved the way for risk-adapted treatment approaches. Although chemoimmunotherapy remains the mainstay frontline treatment, combination therapies incorporating novel agents such as Bruton tyrosine kinase inhibitors, B-cell lymphoma inhibitors, and immunomodulatory agents are being studied, with promising results. Chimeric antigen receptor T-cell therapy and bispecific T-cell engagers have opened a new avenue for treatment. Also promising are antibody-drug conjugates such as ROR1 inhibitors and PI3K inhibitors, which are under clinical investigation. We provide an overview of the molecular mutations identified in MCL and the evolving treatment strategies for this disease.