A 33-year-old man was hospitalized for treatment of a left renal tumor. The radiological findings were consistent with those of a left renal cell carcinoma (RCC). Subsequently, a radical nephrectomy was carried out. Macroscopic examination showed that a well-demarcated tumor measuring 2.9 x 2.6 x 2.5 cm was present in the middle portion of the resected kidney. The cut surface of the tumor was grayish-white in color. Pathological examination of the resected specimen showed a mucinous tubular and spindle cell carcinoma of the kidney (MTSCC-K). MTSCC-K is a low-grade renal epithelial neoplasm that has recently been recognized as a specific entity in the World Health Organization 2004 classification of RCC. To our knowledge, 17 cases of MTSCC-K in Japan have been reported by Japanese investigators. To avoid administration of excessive adjuvant treatment to patients, pathologists and urologists should consider this newly recognized low-grade malignancy when diagnosing renal tumors.