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IntroductionRenal leiomyosarcoma is a rare entity in the world and its understanding is based on reports and various cases; however, the prognosis is bleak for its malignant potential and an average survival of 18 months.Clinical caseA 54-year-old woman with a clinical picture of 6 months of pain in the right flank and a tomographic image of a bilateral renal lesion underwent right radical nephrectomy and left conservative surgery. The definitive histopathological study reported right primary renal leiomyosarcoma with left metastasis.ConclusionRenal leiomyosarcoma is an entity of low incidence and high mortality; however, our case represents the minority of patients with contralateral kidney metastasis reported in the literature.