A 55-year-old male VHL patient with onset of the disease at 42 years of age, genetically diagnosed with a complete allelic loss of VHL. This patient underwent his first surgery in other centre with removal of 2 hemispheric cerebellar Hbs in 2009, requiring a ventriculo-peritoneal shunt due to hydrocephalus. The gene discussed is VHL; the disease is von Hippel-Lindau disease.