Summary

Haemangioblastoma (Hab) is a rare, benign and slow-growing tumour of uncertain histiogenesis. It constitutes roughly 1% to 2% of all intracranial tumours and most commonly arises in the posterior cranial fossa: more than 80% of Habs are located in the cerebellar hemispheres, the remaining cases lie in the vermis, brainstem and spinal cord. In about 20% of the cases Habs are associated with the hereditary disorder known as von Hippel–Lindau disease (VHL). Usually, Hab presents with symptoms of increased intracranial pressure and focal neurological symptoms, which depend on the tumour location. Here, a case of Hab localised in the fourth ventricle that manifested with an unusual clinical picture featuring predominant psychiatric symptoms is described; the condition went unrecognised until the patient’s death.