[Clinical case of the month. Von Hippel-Lindau disease]

VHL disease is a rare genetical disease with a poor long-term prognosis. Clinical features include retineous angiomas, CNS hemangioblastomas, pheochromocytomas and renal cysts. Renal cysts are bilateral and frequently lead to malignant transformation. Surgical treatment of renal cysts is preferably delayed until a minimum of 3 cm diameter has been reached. Treatment has to be as conservative as possible, knowing that uni- or bilateral nephrectomy often has to be carried out later.