The authors report a rare case of primary urethral/paraurethral adenocarcinoma in a female patient. The tumor was first detected at the external and internal urethral orifices, with later recurrences in the region of the urinary bladder neck. Histology did not reveal the exact origin of the malignancy. The patient was treated by transurethral resection, regularly repeated during the 3-6-monthly checks. Five years after diagnosis she remains symptom free and has a high quality of life.