Primary Mullerian duct-type clear cell adenocarcinoma of the urethra is a rare clinical entity with a varied clinical presentation. This can be diagnosed only with a high index of suspicion. Clinical examination, biopsy, and immunohistochemistry are essential for diagnosis. Management will need a multimodal approach with a combination of chemotherapy and surgical excision.
Keywords: adenocarcinoma; female urethra; groin swelling; mullerian duct; urethral cancer.
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