Abstract
We report an extremely rare case of locally advanced prostate cancer in a phenotypic male patient with persistent müllerian duct syndrome. The patient underwent a robot-assisted retzius-sparing radical prostatectomy, bilateral pelvic lymph node dissection, radical hysterectomy, vaginectomy, and salpingo-ophorectomy. He was continent at 3 months follow-up. His follow-up PSA has steadily increased up to 1.4 ng/mL 6 months following surgery and his PSMA-PET scan showed bone metastasis with no local recurrence, and androgen deprivation therapy was started along with docetaxel chemotherapy. Prostate cancer in patients with DSD is extremely rare and can manifest itself with a low PSA, although aggressive cancer.