Abstract: Extranodal NK/T-cell lymphoma (ENKTCL) is a rare subtype of non-Hodgkin's lymphoma with highly aggressive behavior. Its incidence shows significantly geographic and ethnic differences, and is relatively high in Asian countries. Because of high degree of malignancy, rapid clinical progression and poor prognosis of ENKTCL, it is important for patient to receive accurate diagnosis and treatment. To have a better understanding of the biological characteristics and potential targets of novel therapeutic strategies on ENKTCL, this paper expounds the recent progress of ENKTCL in clinical, pathological and genetics features.