[Urologic aspects of Hippel-Lindau disease]

We report on a patient with von Hippel-Lindau disease with special reference to the urological features. Several members of the patient's family had also presented this hereditary disease. Careful follow-up of these patients permits early diagnosis of some of the characteristic tumors. Because it has a tendency to be multifocal and bilateral, surgical treatment of renal carcinoma must be as conservative as possible. For this reason, early tumor excision constitutes the ideal treatment. The importance of family screening and genetic counselling is underscored.