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Von Hippel-Lindau disease is an autosomal dominant cancer syndrome with an estimated prevalence of 1 in 40,000. This disease is not a single entity, but rather a complex of various disorders, some of which are pathologically similar to particular forms of renal cell carcinoma. The clinical diagnosis is made on the basis of the presence of a nephrological or retinal malignant tumor in association with the occurrence of a haemangioblastoma and/or pheochromocytoma. Potential malignancy is assessed after the exclusion of other forms of retinal or renal cell carcinoma. The diagnosis of the other component(s) of the VHL complex depends on the level of screening. Retinal and renal screening for retinal angioma and renal cell carcinoma are the most important parts of the screening programme for VHL. Most patients are adequately screened and carry a life-long risk of developing nephrological or retinal malignant tumors. Neuroendocrinological screening for the appearance of pheochromocytoma should also be undertaken on a regular basis. Pregnancy reduces the risk of developing choriocarcinoma (particularly during the perimortem period) and retinal retinal angioma, and may have a protective role in the development of renal cell carcinoma. Should the patient wish to reproduce, gam

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