Objective To improve the recognition of aggressive natural killer-cell leukemia(ANKL)complicating muhiple organ failure(MOF).Methods A Fare case of ANKL was reported,and the related literatures were reviewed. Results One case of ANKL was diagnosed by bone marrow morphology and immunophenotypes of CD2,CD16,CD56,who developed multiple organ failure involving in liver,kidney,heart,lung,severe metabolic acidosis,tumour lysis syndrome and disseminated intravascular coagulation(DIC)in course of the disease and chemotherapy.VP regimen and symptomatic treatment were performed,and the disease could be stable shortly but died of the failure of lung and heart soon. Conclusion ANKL has a fulminant clinical course and diffuse infihration of tumor cells resulted in multiple organ failure with poor response to treatment and unfavorable prognosis.