Hereditary kidney cancer, also known as familial kidney cancer, is found in only 3% to 4% of cases, and most of these patients have kidney cancer among their fathers or siblings. The most common type of familial kidney cancer is VHL syndrome, which stands for “Von Hippel-Lindau syndrome,” a combination of CNS hemangioblastoma with renal or pancreatic cysts, pheochromocytoma, renal carcinoma, and epidermoid cystadenoma. The average life expectancy of patients with VHL syndrome does not exceed 49 years. The main causes of death are ruptured CNS hemangioblastoma bleeding, renal cell carcinoma and malignant hypertension caused by pheochromocytes. Epidemiological surveys show that the incidence is 1/85,000 to 1/36,000, with a 50% incidence in children of patients, with no significant difference in gender.