Abstract: Primary lymphoma of female genital tract (PLFGT) is uncommon, and its etiology is unclear. It can present with pain, distention, vaginal bleeding, or discharge or be found as an incidental finding at a routine pelvic examination or surgery for other indications. PLFGT can be diagnosed with imaging, with pathomorphology being the key to diagnosing this disease. Further diagnosis and typing are needed with flow cytometry and immunohistochemistry. PLFGT should be differentiated from secondary genital lymphoma and other primary malignant tumors of the reproductive system. Preoperative diagnosis can be challenging due to its rarity. In addition, there is a lack of experience in treating PLFGT. Based on the pathological classification, staging, and non-Hodgkin lymphoma (NHL) guidelines, an individualized approach should be determined. R-CHOP has been found to significantly improve the survival of patients with NHL. However, the efficacy and safety of chemotherapy as the standard treatment for PLFGT still needs to be confirmed. Prognosis was evaluated according to the international prognostic index (IPI).