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OBJECTIVE Tumors in the periurethral area can be a rare clinical entity with many difficulties not only in the diagnosis, as well as in the treatment plan. Skene's gland adenocarcinoma accounts for less than 0.003% of all female urethral malignant neoplasms. CASE REPORT This report describes an extremely rare case of woman with a poorly differentiated carcinoma arising from the periurethral glands. CONCLUSIONS Reporting of such rare cases enhance the understanding of the biological behavior of such tumors and the best treatment plan as