von Hippel-Lindau (VHL) disease is an inherited multisystem familial cancer 
syndrome caused by mutations of the VHL gene on chromosome 3p25. A wide variety 
of neoplastic processes are known to be associated with VHL disease. The 
consequences of the VHL mutations and the pathway for tumor development continue 
to be elucidated. This paper will detail the variety of tumors associated with 
VHL disease and discuss the genetic mechanisms that lead to the predisposition 
for neoplasia.