Polycystic kidneys as the renal lesion in Lindau's disease

Renal malignancy occurring in polycystic kidneys is a rare combination normally thought of as fortuitous. We describe a patient suffering from Lindau's disease, who also had polycystic kidneys and renal carcinoma. On the basis of this case and a review of the literature for similar cases we conclude that the only possibility of outlining a high risk group among patients with polycystic kidneys is to search for other manifestations of Lindau's disease.