Abstract: Nasal and nasal-type NK/T-cell lymphoma is a rare non-Hodgkin's lymphoma arising from putative natural-killer cells. It has distinct clinicopathological features with frequent angiocentricity and angioinvasion accompanied by angiodestruction and zonal necrosis. It can involve almost all of the extra-nodal organs with a highly aggressive course. It has a poor response to treatment because of its multi-drug resistance, which results in an unfavorable prognosis and short life span. There has been no effective treatment until recently. In this article several treatment modalities adopted in recent years have been reviewed and compared, including radiation therapy, conventional chemotherapies, high-dose chemotherapy combined with stem-cell transplantation and L-asparaginase-based induction therapy, and the updated trends are introduced.