Von Hippel-Lindau syndrome (VHL) is a rare genetic disease. Its incidence is 1 : 36,000, there is the familial occurrence in 80 % of cases , the remaining cases are de novo mutations. The disease is caused by the highly penetrant mutations in the VHL gene (3p25.3) and is characterized by the occurrence of benign and malignant neoplasms. The most common VHL tumors are the tumors of the retina, brain and spinal hemangioblastomas, renal cell carcinoma, pheochromocytoma, endolymfatic sac tumors and pancreatic tumors and cysts. The mean age of the VHL patients during the diagnosis is 20-40 years. The diagnosis can be confirmed by a positive family history and the presence of one of the typical tumor. In case of no family history, the diagnosis has to be assessed by the presence of the multiple tumors. The clinical signs and prognosis of VHL depend on the location and extent of the tumors. The life expectancy is 50 years. The most common causes of death are complications of the renal cancer and the brain tumors. The treatment requires a multidisciplinary collaboration through the whole life of patients. This 2 cases report we demonstrate the differences among the patients with de novo mutations disease and the patient with familial incidence.Key words: pheochromocytoma - renal cell carcinoma - von Hippel-Lindau syndrome.