Von Hippel-Lindau (VHL) disease is a progressive autosomal dominant multisystem disorder that is associated with a germ line mutation of the VHL gene on the short arm of chromosome 3. A variety of benign and malignant diseases, including eye and CNS hemangioblastomas, renal cell carcinoma and pheochromocytoma are the major components. Gastroenteropancreatic neuroendocrine tumors are also listed among the typical complications, although these occur seldom. Virtually all such tumors are pancreatic islet cell tumors. VHL-associated islet cell tumors are mostly hormone-inactive. They can be detected during screening investigations according to the multidisciplinary disorder or by workup of space-occupying lesions. There are no specific predictors for malignancy in VHL-associated islet cell neoplasias, but tumors smaller than 3 cm in diameter are believed to be always benign. Gadolinium-enhanced MRI is currently the imaging method of choice, but contrast-enhanced CT is also a diagnostic option. The spectrum of manifestations is illustrated by selected cases.