Synovial cell sarcoma (SCS) of the kidney is a rare tumor entity with a poor prognosis. Morphologic and immunohistochemical characteristics may overlap with other more common neoplasms of the kidney. Therefore, the diagnosis of primary renal SCS not only requires the exclusion of similar tumor types, but also a confirmation of SYT-SSX gene fusion using molecular techniques. The treatment comprises radical surgery, and, depending on age and health status, adjuvant chemotherapy in selected patients. Here, we present an elderly SCS patient in whom straightforward radical surgical treatment resulted in a sustained complete remission; it allowed us to perform a literature survey focusing on current diagnostic tools for SCS.