Patients with von Hippel-Lindau disease (VHL) often harbor significant disease 
burden within the CNS, specifically craniospinal-axis hemangioblastomas and 
endolymphatic sac tumors (ELSTs). The majority (60-80%) of patients with VHL 
harbor hemangioblastomas, and 10-15% will develop ELSTs. Advances in the 
understanding of the natural history and outcomes associated with the surgical 
management of VHL-associated tumors have led to improved management of patients 
with VHL. Optimizing indications for surgical intervention and refining of 
surgical techniques for these lesions can reduce patient morbidity associated 
with the management of this syndrome. In this article, we review the various 
aspects of perioperative management of patients with VHL, surgical indications 
and general operative principles for the management of hemangioblastomas and 
ELSTs, and outcomes associated with the surgical treatment of these tumors.