Renal Leiomyosarcoma (LMS) is an exceptionally rare and aggressive form of RCC (renal cell carcinoma), comprising of 0.5-1% of all adult RCC. This disease constitutes a 5-year 25 % overall survival. We present a rare case of renal leiomyosarcoma in a 58 year-old Caucasian female with final pathologic staging of pT2 who underwent robotic-assisted right partial nephrectomy. Despite negative margins, given her aggressive histology, adjunctive treatment is discussed.
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