Von Hipple Lindau disease (VHL) is an autosomal dominant syndrome caused by a mutation of the VHL tumor suppressor gene, inducing various benign and malignant tumors of the central nervous system, renal carcinomas or cysts, pheochromocytomas, epididymal cystadenoma and pancreatic tumors and cysts [117]. Here, VHL is linked to pancreatic neoplasm.