A 79-year-old man diagnosed with VHL disease 20 years ago when, during an ophthalmological examination, capillary haemangiomas were found in both eyes (AO). He has seven children, three of whom and one granddaughter are affected by the disease. The only systemic involvement of the disease is renal cysts. She currently has a prosthesis in her right eye (OD) due to a former tractional retinal detachment (RD), and in her left eye (OI) she has a visual acuity (VA) of 0.3 with nasal photocoagulated haemangiomas and another larger one below, together with an epiretinal membrane in the posterior pole that alters the normal macular anatomy, compatible with the upper hyperreflective line shown in the optical coherence tomography (OCT). The membrane appeared after a vitrectomy performed 2 years ago after the patient presented with a grade III vitreous haemorrhage and an inferior tractional RD. After vitrectomy, the vitreous haemorrhage persisted and was treated with an intravitreal injection of anti-VEGF (bevacizumab), which resolved the haemorrhage.