Von Hippel-Lindau syndrome is an autosomal dominant inherited phacomatosis with 
a predisposition for the central nervous system and retina. There is variable 
expression with hemangioblastomas in the brain, medulla oblongata, spinal chord, 
renal carcinoma, pheochromocytoma, pancreatic cysts and islet cell tumors as 
well as tumors of the endolymphatic sac of the inner ear. Clinical symptoms 
occur first after an age of approximately 30 years. Magnetic resonance imaging 
(MRI) of the neuroaxis is indicated in all patients with a suspicion of von 
Hippel-Lindau syndrome.