Abstract We report a 39-year-old woman with clear cell adenocarcinoma of the urethra (CCAU). She was treated with surgery alone and has survived 8 years without recurrence. The patient had painless hematuria. Imaging showed a 5.1 cm urethral mass. The tumor invaded the bladder and vagina (clinical stage T3N2M0). Urine cytology confirmed the diagnosis, which is unusual for CCAU. She underwent laparoscopic radical cystectomy, lymph node dissection, and cutaneous ureterostomy.
