Von Hippel-Lindau (VHL) disease is a hereditary tumor syndrome that can present as angioblastoma of the cerebellar crestal medulla, retinal hemangioma, renal tumor cyst, pancreatic tumor cyst, adrenal pheochromocytoma, inner ear lymphoma, and epididymal cystadenoma. In 1927, the Swiss pathologist Lindau reported that retinal hemangiomas were associated with cerebellar hemangiomas. In 1964, Melmon first named this familial hereditary tumor syndrome, Von Hippel-Lindau disease, after two medical experts, and it was widely recognized. 1993, Lalif et al. In 1993, Lalif et al. studied the VHL family and identified the VHL gene at 3P25-26 and successfully cloned the VHL gene for the first time, which greatly promoted the study of Von Hippel-Lindau disease. Foreign statistics show that the incidence of VHL disease is 1:36,000; VHL disease patients have different epizootic rates and degrees of manifestation, with age-dependent epizootic rates of 80-90% up to age 65; renal cancer and central nervous system hemangioblastoma are the main causes of death. Diagnostic criteria for Von Hippel-Lindau disease: For patients with a family history of VHL, the diagnosis is made by the presence of any one of retinal hemangioma, CNS angiogenic tumor, renal carcinoma, or pheochromocytoma. For sporadic patients, the presence of one retinal or CNS angiogenic tumor and one substantial organ tumor is also diagnostic. For those who are clinically suspected but do not meet the above diagnostic criteria, VHL genetic testing is very important. Since the discovery of the first VHL disease kidney cancer family in 2001, the Department of Urology of Shanghai Renji Hospital has identified more than 20 VHL disease families and systematically carried out VHL gene testing and minimally invasive treatment of renal cancer and adrenal pheochromocytoma, with very good results.