The association of von Hippel-Lindau disease and renal call carcinoma is well recognized. However, the most appropriate surgical approach in patients with von Hippel-Lindau disease and renal carcinoma remains controversial. We examine this controversy and report on our experience. Our cohort consisted of 28 affected members of three von Hippel-Lindau kindreds. Screening of these individuals consisted of an abdominal ultrasound (US) in their early teens and a baseline computerized axial tomography (CT) scan in their late teens. To date, nine patients have been found to have renal cell carcinoma. In three of these patients, the surgical specimen revealed multiple small foci of "clear cells" characteristic of renal cell carcinoma that had not been detected by careful radiological assessment prior to surgery. In two patients, these lesions were located at the opposite pole from the radiologically-detected tumor. Nephron-sparing surgery, therefore, risks leaving potential tumor that may be responsible for mortality. Conversely, radical procedures condemn a patient to life-long dialysis and/or transplantation with associated risk and morbidity. The potential for the metachronous emergence of contralateral renal cell carcinoma, however, justifies efforts to preserve normal tissue in patients harboring radiologically-detected lesions; however, we need to know with some certainty the natural history of the correct management of this disorder.