OBJETIVE: Primary undifferentiated pleomorphic sarcoma (UPS) of the testicular tunics is rare, and synchronism with other malignancies of the urinary tract is uncommon, and may complicate the staging and therapeutic approach. We report the case of a patient diagnosed with primary paratesticular UPS with synchronous Renal Carcinoma. METHODS: Patient presenting with intrascrotal tumor who underwent left radical orchiectomy. In staging work up studies a second urologie neoplasia was found in the kidney. Histological diagnosis using immunohistochemical techniques for adequate characterization was performed. Adjuvant treatment options were evaluated. We review the literature and discuss the case. RESULTS: The UPS diagnosis was performed with immunohistochemistry. Paratesticular origin from testicular tunics was evident in the macroscopic evaluation. After finding the synchronic ipsilateral renal tumor, he underwent conventional left radical nephrectomy, ipsilateral adrenalectomy, excision of remnant left cord and para-aortic and pelvic lymphadenectomy. He received chemotherapy and adjuvant radiotherapy. Currently, after twelve months of follow-up there is no evidence of disease. CONCLUSIONS: The UPS is a heterogeneous group with an exclusion immunohistochemical diagnosis. Its management requires a multidisciplinary approach; the initial surgical treatment is accepted for the paratesticular mass with high section radical orchiectomy. Although there is insufficient evidence of the efficacy of adjunctive lymphadenectomy, chemotherapy and/or radiotherapy.