Context: Clear cell adenocarcinoma of the urethra is an extremely rare tumour. Its histogenetic derivation remains controversial.
Case report: We report a new case of clear cell adenocarcinoma of the proximal urethra in a 56-year-old woman who presented with grossly hematuria. Urethral cystoscopy revealed a tumour protruding from the posterior urethral wall at the bladder neck. Treatment consisted of urethrocystectomy with pelvic lymph node dissection. Histologically, the neoplasm consisted of clear cell adenocarcinoma of the urethra.
Conclusion: It appears that female urethral adenocarcinoma has more than one tissue of origin.
Keywords: Clear cell carcinoma; histopathology; urethra.