A case of leiomyoma of the kidney is reported. A 57-year-old man with the chief complaint of right back pain was consulted to our hospital. Abdominal computed tomography (CT) demonstrated a right renal tumor. Right renal angiography revealed a hypovascular lesion. Under the diagnosis of right renal tumor, right radical nephrectomy was performed. Gross evaluation revealed a well encapsulated solid mass. The tumor was 48 x 43 x 42 mm in diameter and a hemorrhagic region was present. Microscopically, the tumor was composed of interlacing bundles of spindle cells. Diagnosis was leiomyoma of the kidney. We reviewed 42 cases of renal leiomyoma reported in Japanese literature.