A 26-year-old man with a decrease in visual acuity (VA) in the left eye (LA) of two years' evolution. Carrier of the causal mutation of VHL disease, father and brother with systemic manifestations of the syndrome. On examination, the VA of the LA is 0.4. The left fundus showed an orange endophytic tumour of two papillary diameters in the superior temporal arcade with dilated nutrient vessels. Optical coherence tomography (OCT) showed diffuse cystic oedema in the posterior pole and angiography (AFG) showed early hyperfluorescence of the lesion with late leakage. A systemic study was requested, which also showed multiple solid nodules in both kidneys suggestive of bilateral hypernephroma (later confirmed after radical left nephrectomy), a hypercapillary nodule in the right adrenal gland, a probable pancreatic neuroendocrine tumour and multiple haemangioblastomas in the pons and spinal cord, with mild associated medullary oedema.

Argon laser photocoagulation was started in two sessions during which subretinal haemorrhage along the vascular arcade and vitreous haemorrhage (VH) occurred. Two months later, in the absence of reabsorption of the VH, a 23G vitrectomy was performed, during which treatment of the lesion was completed using an endolaser. Three months after surgery, VA was 0.6. OCT reveals a good foveal profile, with resolution of macular oedema. The AFG confirms the closure of the nutritional vessels, the lesion has a cicatricial appearance, and although it captures early on, there is no leakage, with replacement vessels being seen in the periphery of the lesion.