A 62-year-old man, who complained of gross hematuria, was admitted because of a space occupying lesion of the right kidney and left non-visualizing kidney on IVP. The right pyelogram was compressed laterally, and the left pyelogram showed a moth-eaten appearance of the middle and lower calyces which was consistent with tuberculosis. Abdominal aortography revealed a hyperneovascularization over the middle and upper portions of the right kidney. CT-scan revealed a low density area of the right kidney and left atrophic kidney. No distant metastases were disclosed by bone scanning, liver scanning, lymphangiographic and chest X-ray studies. Bilateral nephrectomy was performed because the right renal tumor occupied the main renal artery. Hemodialysis was started on the next day. Postoperative course was uneventful. Histological examinations of the surgical specimen revealed a right renal adenocarcinoma and left renal tuberculosis. We discuss the pathogenesis and treatment of the combination of renal tuberculosis and malignant tumor.