NK/T cell lymphoma, a rare type of non-Hodgkin's lymphoma, is a highly aggressive disease with poor prognosis. Clinically, it is classified into nasal, non-nasal, and aggressive lymphoma/leukemia subtypes. They are characterized by geographic distribution and are universally associated with Epstein-Barr virus (EBV) infection. Due to its low occurrence and dismal clinical outcome, no therapeutic strategy is currently identified in this disease. Combined chemotherapy and radiotherapy have better effects for stage I/II nasal NK cell lymphoma. As for stage III/IV nasal NK cell lymphoma and non-nasal, and aggressive subtypes, chemotherapy is the main treatment method. Recently, some studies have demonstrated promising outcomes in the selected cases by high-dose chemotherapy supplemented with auto- or allo-HSCT.