Renal leiomyoma is a benign neoplasm that has been reported frequently in uterus but rarely in kidney. The aim of this study is to show a case report of primary renal leiomyoma and review literature. A 59-year old female patient presented with an indolent right kidney mass. AbdominalCT Scan showed a well-defined hyperattenuating solid mass, no invade. Microscopically, the lesion showed typical features of leiomyoma, including spindle cells with deeply eosinophilic cytoplasm, arranged in intersecting fascicles. Thetumor cells were diffusely positive with vimentin, SMA and desmin.