Abstract: The case of uterine natural killer/T-cell lymphoma combined with hemophagocytic syndrome is very rare in clinic. It is a serious and aggressive disease with rapid progress and high mortality. In addition, it is difficult to differentiate the disease from other malignant tumors of the reproductive system due to the non-specificity of the related auxiliary examination results and clinical manifestations, leading to a high misdiagnosis rate. To reduce misdiagnosis and missed diagnosis of the disease and improve the prognosis, the case data of a patient treated by the Central Hospital of Enshi Tujia and Miao Autonomous Prefecuture in March 2020 is introduced, and the methods of diagnosis and treatment are discussed.

Key words: Genital neoplasms, female;, Natural killer/T-cell lymphoma, Lymphoma, T-cell;, Killer cells, natural;, Uterus, Lymphohistiocytosis, hemophagocytic, Case reports