Von Hippel-Lindau disease associated with an invasive choroid plexus tumour presenting as a middle ear mass

Cerebellar haemangioblastomata and angiomata of the retina are the most common vascular tumours seen in von Hippel-Lindau disease. A definite association between this condition and choroid plexus tumour has not been described previously and its presentation as a middle ear mass is unique.