Persistent Mullerian duct syndrome.

Persistent Müllerian Duct Syndrome is a rare genetic disorder, consisting in the persistence of the müllerian structures in males. We report the case of a 29-year-old male patient presented with an abdominopelvic mass. Radiological examination showed a 30cm abdominopelvic mass that was biopsied, and the pathological examination concluded to a testicular seminoma. The patient underwent surgery after neoadjuvant chemotherapy, where a uterus with right adnexa were discovered. This case highlights the therapeutic challenges of persistent Müllerian duct syndrome and the importance of the early diagnosis to optimize fertility and reduce the risk of testicular degeneration.