Objective To analyze and explore the clinical and pathological characteristics of primary endometrial carcinoma of the ovary and uterus,and review the literature in order to improve the understanding and diagnosis of this type of disease.Methods This article reviews a case of double primary endometrioid carcinoma of the ovary and uterus treated at the Xinglin Branch of the First Affiliated Hospital of Xiamen University for clinical and pathological analysis,and reviews relevant literature to summarize the latest progress in diagnosis and treatment at home and abroad.Results The case was confirmed by histopathological and immunohistochemical staining results.Endometrioid carcinoma with characteristic pathological morphology is observed in the ovaries and uterus.The immunohistochemical results showed that ER,PR,Vim,and PAX8 were all expressed positively,while P53,P16,and NapsinA were expressed negatively.Conclusion The clinical and imaging manifestations of this type of disease are specific,often accompanied by endometriosis.The main treatment method is comprehensive staged surgery,and the prognosis is good.