Introduction and importance: The coexistence of a benign Brenner tumour and a mucinous cystadenoma is rare, and their relationship and origin are still enigmatic and challenging. Case presentation: In this manuscript, the authors report a case of a 62-year-old nulliparous Syrian woman who presented with severe abdominal distension, which was followed by laparotomy and the excision of a 25×20 cm-cyst; its pathological examination confirmed a benign Brenner’s tumour and mucinous cystadenoma. Clinical discussion: Ovarian Brenner and mucinous are usually benign and can rarely grow asymptomatically to very big sizes. Herein, the authors aim to emphasize the importance of excluding malignancy by pathology examination. Conclusion: Walthard cell nests undergo metaplasia to give rise to different kinds of Brenner and mucinous neoplasm formations according to their genetic alterations. This paper adds to the available literature, which is still poor, by providing evidence of the first case of this rare combination from Syria with a review of different theories of origin and differential diagnoses. More studies directed towards exploring this combination’s genetic origin are needed to boost our understanding of ovarian tumours in general.
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