Abstract: Von Hippel-Lindau (VHL) syndrome, also known as cerebral retinal angiomatosis, is a kind of neuroendocrine tumor. The incidence rate is high, and the heredity is very high, which can involve the retina, central nervous system, various organs and various tissue parts. This paper reports a case of VHL syndrome with hypertension and diabetes as the main manifestations, in order to improve the understanding of the disease and reduce misdiagnosis and missed diagnosis.