Abstract: Extranodal natural killer (NK)/T- cell lymphoma, nasal type (ENKL) is an uncommon subtype of non- Hodgkin's lymphoma, which is aggressive and has poor prognosis. ENKL occurs predominantly in the nasal cavity and less frequently in the skin and in the gastrointestinal tract. The pathology of this disease is characterized by vascular invasion and destruction. ENKL is strongly associated with the Epstein–Barr virus, which aids in the diagnosis of ENKL. The characteristic markers on the surface of an NK/T cell and the spe -cific genetic variations are also useful in the diagnosis. At present, the treatment of ENKL is still under discussion. Although radiothera -py combined with chemotherapy on patients with early-stage ENKL and L-ASP-based chemotherapy and hematopoietic stem cell trans-plant on patients with advance-stage ENKL have achieved favorable effects, further studies are still necessary to develop the principles and methods of a standardized treatment.