Von Hippel Lindaus(VHL) disease is an autosomal dominant syndrome with 1 of 36 000 individuals affected. It´s caused by different types of mutations in the von Hippel Lindau gene located on the short arm on chromosome 3. The syndrome involves malignant and/or benign neoplasia in retina, cerebellum, brainstem, spinal cord , pancreas, epidydimis, the broad ligament of the uterus and – most often renal cell carcinoma. The risk of developing some of the tumours shows a genotype-phenotype correlation. Renal cell carcinoma are responsible for 50% of all deaths, caused by the VHL syndrome. Unfortunately kidney cancer is chemoresistant, and therefore the best treatment is early surgery , which is the only possibility for permanent cure. To detect tumorurs as early as possible screening and close follow up are recommended in individuals at risk. Several studies are performed to unravel the molecular defects involved, and hopefully this will be helpful in the development of new effective drugs against kidney cancer. In the present thesis an overview of different cellular pathways, disturbed in VHL patients, are described. Possible etiological intracellular mechanisms, involved in carcinogenesis in VHL syndrome, is focused, assessing the biological significance of each pathway, and the possible interactions between them. Finally, the search for targeted therapy against tumour cells, based on basic biological understanding, is investigated, and the need of future investigational break through is underlined.