Urethral cancer in females is a rare occurrence. Although initially believed to be derived from skene glands, there is no consensus on the cell of origin. Squamous cell carcinoma is the most common, whereas adenocarcinoma is the least common. We report the case of a 58-years-old lady with a history of voiding lower urinary tract symptoms, later requiring a suprapubic cystostomy for retention. Cystoscopy and biopsy showed prostate-specific antigen (PSA) negative adenocarcinoma and postiron emission computed tomography (PETCT) showing pelvic and inguinal nodal metastasis. The patient underwent neoadjuvant chemotherapy followed by anterior exenteration with pelvic lymphadenectomy and ileal conduit. Final histology showed ypT4 yN1 adenocarcinoma, not otherwise specified.
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