A 58-year-old woman was seeking medical aid because of a dense mass in her groin that had grown during the previous 3 weeks. Biopsy of the lymph node showed keratinizing squamous cell carcinoma whose primary origin was unknown. Therefore, pelvic and inguinal lymph node radiation therapy was provided. One year after the first symptom, a 1 cm polyp was detected in the urethra, and histology showed squamous cell carcinoma. The patient underwent a total urethrectomy. As epicystostomy caused intolerable irritation for the patient she underwent a cystectomy with ileal conduit. Four months later CT showed progression of the disease and the patient started receiving palliative chemotherapy. Currently the disease remains controlled. Primary urethral carcinoma (PUC) is a rare disease, accounting for less than 1% of genitourinary cancers. Because of its rarity, there are no standardized treatments for PUC and available treatments can vary between different institutions. However, the strategy in the treatment of PUC is generally determined by the gender of the patient, and the stage and location of the tumour.