Coexistence of renal cell carcinoma and renal artery disease is an unusual and challenging problem. From 1969 to 1991, 34 patients presented with localized renal cell carcinoma and renal artery disease affecting all of the functioning renal parenchyma. These patients represented 4 categories: 1) a solitary kidney with renal cell carcinoma and renal artery disease (5), 2) bilateral renal cell carcinoma and coexistent renal artery disease (5), 3) unilateral renal cell carcinoma and contralateral renal artery disease (13), and 4) unilateral renal cell carcinoma and bilateral renal artery disease (11). Atherosclerosis was the most common cause of renal artery disease (30), followed by medial fibroplasia (2), renal artery aneurysm (1) and arteriovenous malformation (1). A total of 23 patients (68%) presented with azotemia (serum creatinine 1.5 mg./dl. or more) and 11 (32%) presented with hypertension. All patients underwent complete surgical excision of renal cell carcinoma. A nephron sparing operation was performed preferentially (30 patients) and bilateral renal cancer operations were staged. Eight patients underwent simultaneous partial (6) or radical (2) nephrectomy and surgical renal revascularization. There were no operative deaths. Postoperatively, preservation of renal function was achieved in 33 patients and 1 required chronic dialysis. At mean followup of 47 months 23 patients (68%) were alive with no evidence of malignancy and 2 were alive with recurrent renal cell carcinoma. Three patients died of metastatic renal cell carcinoma, while 6 died of unrelated causes. All of the latter 6 patients were free of renal cell carcinoma at death. Nephron sparing surgery combined occasionally with renal arterial reconstruction can yield gratifying results in this complex patient population.