A case of nephrogenic adenoma in the female urethral diverticulum is reported. A 39-year-old woman visited our hospital with the chief complaint of perineal pain. Observation of her perineum and transvaginal digital examination revealed urethral diverticulum, and then diverticulectomy was performed. Histological diagnosis was urethral diverticulum associated with nephrogenic adenoma. In Japan, this is the 1st report of nephrogenic adenoma arising in the female urethral diverticulum. We reviewed 305 cases of nephrogenic adenoma of the urinary tract in the English and Japanese literature.