Clear cell adenocarcinoma of the female urethra is extremely rare. We herein describe the 33rd case of clear cell adenocarcinoma of the female urethra in Japan. A 54-year-old female who presented with pollakisuria was referred to our department. Transvaginal examination showed a walnut-sized firm mass on the anterior vaginal wall. Computed tomography, magnetic resonance imaging (MRI), cystourethroscopy and the histopathological findings of the biopsied specimen revealed adenocarcinoma of the urethra. Anterior pelvic exenteration and ileal conduit urinary diversion were performed and the final pathological diagnosis was clear cell adenocarcinoma of the urethra, pT3, pN2. No further adjuvant therapy was conducted. She remains alive 6 months after surgery in spite of paraaortic and inguinal lymph node metastases.