Abstract: Objective : To investigate the clinical characteristics, treatment outcomes, and prognostic factors of patients with primary female genital system lymphoma (PFGSL). Methods : A total of 25 cases of PFGSL seen inour hospital from January 1976 to August 2006 were analyzed retrospectively. The clinical data including clinical stage, grade, treatment and prognosis were analyzed．Results: The mean age was 41.2 years. The lesions of PFGSL mainly involved ovary (11 patients), cervix (7 patients), uterus (5 patients) and vagina (2 patients). PFGSL presented with a broad range of pathologic types. Twenty-two cases originated from B-cells, 1case originated from T-cells, and 2 cases were indefinite in origin. According to the International Working Formulation (IWF) guidelines, 78.3% were low to intermediate grade and 21.7% were high grade. The Ann Arborstaging included stage ⅠE (32.0%), stage ⅡE (24.0%), stage ⅢE (20.0%) and stage ⅣE (24.0%). Most patients received comprehensive treatment including surgery, chemotherapy and/or irradiation. Of these 25 cases, 3 received chemotherapy and radiotherapy after surgery, 18 received surgery and chemotherapy and 2 received chemotherapy and radiotherapy. Only 2 cases were treated with surgery alone. The median follow-upperiod was 25 months. The 5-year overall survival rate was 32.0%. The originally involved organs, Ann ArborStage, and IWF were significant prognostic factors for survival, with a statistical significance. Conclusion : Withno typical clinical symptoms, PFGSL has a poor prognosis. Surgery and chemotherapy play a very importantrole in the comprehensive treatment. Significant prognostic factors for survival are Ann Arbor stage, IWF andthe originally involved organs.