Twenty-two cases of adenocarcinoma of the urethra in women were studied. Nine were classified histologically as clear cell adenocarcinoma and 13 were classified as columnar/mucinous adenocarcinoma. Thirteen patients (59%) were black. The average patient age was 61 years (range, 27 to 76 years). Follow-up ranged from 6 to 194 months, (average, 40 months). At presentation, most patients (82%) had extension of tumor into adjacent structures or metastases to regional lymph nodes. Eighty-six percent received radiation therapy and 41% underwent an anterior exenteration or cystectomy. Eight of 22 patients (36%) had no evidence of disease 21 to 194 months after diagnosis (average, 83 months). Fourteen (64%) were dead of disease 6 to 23 months after diagnosis (average, 15 months). In general, the extent of tumor correlated best with survival time. Forty-four percent of patients with clear cell adenocarcinoma were dead of disease within 24 months of diagnosis, in contrast to 77% of those with columnar/mucinous adenocarcinoma, suggesting that patients with clear cell adenocarcinoma may have a better prognosis than those with columnar/mucinous adenocarcinoma. However, the difference in survival probability between the two groups was not found to be statistically significant. Recognition of the two histologic types of urethral adenocarcinoma is important to prevent misdiagnosis of such tumors as metastases (or direct extension) of nonurethral neoplasms having a similar histologic appearance. A possible predilection of the disease for black women has not been previously described.