Malignant Brenner tumor (MBT) of the ovary is a rare malignant ovarian tumor, whereas uterine corpus endometrioid carcinoma (UEC) constitutes one of the most common malignant tumors of the female reproductive system. The present study reported on a case of the coexistence of ovarian MBT and borderline mucinous cystadenoma combined with primary UEC. Therefore, the present case is a synchronous primary cancer of both ovary and endometrium. Although synchronous primary cancers of the endometrium and ovary are relatively uncommon, they are not rare; however, due to the rarity of MBT, this case was considered singular. To the best of our knowledge, this was the first-ever reported case of the coexistence of an ovarian MBT and borderline mucinous cystadenoma combined with primary UEC. Based on a review of the literature associated with the present case, its clinicopathological features, immunohistochemical phenotype, differential diagnosis, molecular changes, prognosis and treatment were summarized and discussed. The aim of the present study was to improve the understanding of this rare synchronous primary cancer of the ovary and endometrium so as to avoid future misdiagnosis.