This is a case report of a rare tumour of the ovary originally developing from the embryonal vestiges of the Wolffian duct and becoming a pure mesonephrotic carcinoma. Although initially considered to be benign, the course of the tumour is one of a potential low grade long term malignancy. Histological changes are of a pure mesonephrotic carcinoma or of a female adnexal tumour probably of wolffian origin (FATWO). It showed few specific traits. Each site of the development is constantly linked to the anatomy of the Wolffian duct. Metastases and recurrencies can develop late (after 5 to 10 years) following radical surgery. A survey of the literature confirmed the salient features of these tumours, which can differ from those of Müllerian origin.
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