Introduction: Von Hippel-Lindau disease (VHL) is autosomal dominant tumor syndrome that debuts mostly in young adults , patients with this disease are linked to the triggering of various types of benign and malignant neoplasms in multiple locations, systems and organs, in particular affecting more the nervous system and other internal organs. Approximately this tumor syndrome shows an incidence rate of 1 in 36,000 live births with a penetrance greater than 90%. The molecular basis of VHL disease is the impairment of VHL protein function and the consequent clustering of hypoxia-inducible factors with subsequent consequences on cell differentiation and metabolism. Objective: to present current information related to Von Hippel-Lindau disease, description, genetics, molecular basis, classification and manifestations of the disease. Methodology: a total of 33 articles were analyzed in this review, including review and original articles, as well as clinical cases, of which 26 bibliographies were used because the other articles were not relevant to this study. The sources of information were PubMed, Google Scholar and Cochrane; the terms used to search for information in Spanish, Portuguese and English were: Von Hippel-Lindau, VHL, tumor suppressor gene, pheochromocytoma, hemangioblastomas. Results: VHL has an incidence of approximately 1 in 36,000 live births, with a penetrance of over 90%. These present tumors are initiated by inactivation of biallelic VHL and are related to pathologic activation of hypoxic gene response pathways. Conclusions: Von Hippel-Lindau disease is an autosomal dominant disorder that is generated by mutations in the VHL tumor suppressor gene. Within the field intrafamilial variation may evidence correctly shaped genotype-phenotype connections for renal cancer and pheochromocytoma risks. Visceral cysts (renal, pancreatic and epididymal) are frequent, however organ function involvement is rare. They usually occur with hemangioblastomas of the central nervous system and retina, as well as renal cancers. Unusually it includes non-functioning pancreatic endocrine cancers, adrenal and extra-adrenal pheochromocytomas, endolymphatic sac tumors, as well as head and neck paragangliomas.