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Background: Renal leiomyoma is a challenging diagnostic and therapeutic condition. Given that 90% of leiomyomas originate from the renal capsule, leiomyoma presenting as a renal parenchymal mass is extraordinarily rare. Case Presentation: Herein, we report the clinical and imaging features of a patient with renal leiomyoma occurring in the renal parenchyma and mimicking renal cell carcinoma. We also review the clinical, imaging, and histological features of renal leiomyoma. Conclusion: An initial partial, simple or radical nephrectomy according to tumor size and patient’s underlying condition is suitable for larger, heterogeneous, and non-peripherally located tumors, even if they demonstrate hypointensity on T1- and T2-weighted images, considering the possibility of other diagnoses.