A 49-year-old female visited our hospital because of bloody discharge from the urethra. On examination, an elastic-soft mass was palpable beneath the anterior vaginal wall. The vaginal mucosa was intact. Cystourethroscopy demonstrated neither tumor nor diverticular orifice. Pathological specimen obtained by needle biopsy revealed clear cell adenocarcinoma. The patient underwent anterior pelvic exenteration and modified Kock pouch urinary diversion. The microscopic appearance suggested that the tumor arose from the paraurethral duct. Stains for prostatic specific antigen and prostate specific acid phosphatase were negative. Forty cases of clear cell adenocarcinoma of the female urethra reported in the literature were reviewed.