Paravaginal female adnexal tumor of probable wolffian origin

Female adnexal tumor of wolffian origin (FATPWO) is a lesion that predominantly occurs in the broad ligament. Its occurrence in the right paravaginal area is rare; only one other case has been reported to occur at this site. The authors report a case of paravaginal FATPWO in a 20-year-old woman that recurred twice during a 5-year period. The microscopic and ultrastructural features of this lesion were identical to other cases of FATPWO reported in the literature. This tumor must be recognized, especially at this site, so that it is not confused with vaginal adenocarcinoma related to diethylstilbestrol exposure in utero.