Primary renal malignant fibrous histiocytoma is a rare tumor of the kidney. We report an additional case noting, as other authors have, the similarity in clinical presentation with renal cell carcinoma. We also review the 23 documented cases of the international literature, emphasizing the current diagnostic approaches and therapeutic management.

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We report a case of a 69-year-old man with a history of left spermatic cord thickening and an enlarging painless mass in the left hemiscrotum. A final diagnosis of paratesticular epithelioid leiomyosarcoma was made. 13 months after treatment he is alive without any signs of tumor recurrence or metastasis.

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