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Introduction: Brenner tumor of the ovary represents an uncommon neoplasm. It constitutes about 1.5% of all ovarian tumors with a peak of incidence in the fifth decade. The vast majority of Brenner tumors is benign, while only 1% is malignant and associated with a poor prognosis. Diagnosis could be made only with histological examination, and surgery is the mainstay of treatment. Materials and methods: Here we present the case of a patient affected by a malignant Brenner tumor of the ovary, with metastases to brain, lung and liver and by a concomitant low grade endometroid adenocarcinoma of the uterus. A postoperative platinum based chemotherapy (CHT) was planned. The adjuvant treatment was discontinued for hematologic and liver toxicity and the patient died one month later due to a cardiorespiratory arrest. Results and discussion: For what concerns adjuvant therapy, it is supposed that chemotherapy is profitable in the survival of these patients, but what agents would really provide an objective response in the presence of metastatic disease is not standardized. Conclusion: Because of the rarity and the limited good quality research reports, multicentric prospective trials are needed to improve preoperative diagnosis of MBTs and investigate the best treatment. more...