A case of Bellini duct carcinoma is reported. A 71-year-old woman visited our hospital with a chief complaint of lower abdominal pain. Computerized tomography, ultrasonography showed a mass lesion measuring about 5 cm in the right kidney. Angiography showed an avascular mass lesion in the right kidney. Right radical nephrectomy was performed under the diagnosis of renal cell carcinoma. Histological examinations showed Bellini duct carcinoma of the papillary type. We performed M-VAC (methotrexate, vinblastine, doxorubicin, icisplatin) therapy as is used for transitional cell carcinoma. She is alive with no evidence of disease 5 months after her surgical treatment. To our knowledge, only 32 cases of Bellini duct carcinoma have been reported in the Japanese literature.