Extranodal natural killer/T-cell lymphoma, nasal type (ENKL), is a rare subtype of non-Hodgkin lymphoma, and its treatment outcome was previously poor. Novel treatment strategies have improved the outcomes of ENKL remarkably in the last decade. Nowadays, patients with localized nasal ENKL are recommended treatment with concurrent chemoradiotherapy, and their 5-year overall survival rate is approximately 70%. In patients with advanced or relapsed/refractory disease, the efficacy of L-asparaginase containing therapy has been confirmed. However, there still remain unmet needs in the treatment of ENKL. Continued efforts should be made to further improvements in the treatment of ENKL.