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Renal leiomyoma is a benign finding in kidney pathology. It has been documented in various organs; renal location is less frequent and has been rarely documented in the literature. We present here the case of a renal leiomyoma revealed by an abdominal mass and flank pain. The diagnosis of certainty is histological, generally on surgical specimens. Due to the challenges associated with clinically diagnosing this tumor, a high level of suspicion is warranted when a patient presents with sizable and clearly defined renal lesions., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Nassira et al.)