Introduction: Von Hippel-Lindau disease (VHL) is a multi-systemic disease characterized by abnormal multiplication of blood vessels, with haemangioblastomas and cysts, as well as other neoformations in the blood Several organs. We report the case of a patient; we try to put the point on this pathology. Case Report: 14-year-old patient, consults for a BAV of the OD whose examination finds an intra vitreous haemorrhage,retinal capillary haemangioma and a lower exudative retinal detachment. The patientreceived an IVT of anti VEGF initially followed by an endocular surgery. Postoperative visual acuity was not improved with a follow-up of 6 months. Discussion: The clinical definition of VHL disease is based on the presence of two major lesions, one of which is hemangioblastoma. The absence of family history, or a single injury in the presence of a family history. Complementary examination is based on angiographyto fluorescein. Complications can range from simple exudates to DR or even phtyse of the globe. The treatmentis essentially based on argon laser coagulation, cryotherapy, anti-VEGF and retinovitealstage of complications. Conclusion: VHL is a hereditary disorder with multi-systemic involvement, hence the interest of an examination complete follow-up, regular monitoring and genetic counseling in any patient suspected of having the diseaseas well as his family