Von Hippel-Lindau (VHL) disease is a multisystem genetic disease, the cardinal 
manifestations of which include central nervous system hemangioblastomas (CNS 
HB), renal cell carcinomas (RCC), and pheochromocytoma. Tumorigenesis in VHL of 
both RCC and CNS HB occurs secondary to downstream effects of a mutated or 
absent VHL protein. Treatment of RCCs with tyrosine kinase inhibitors (TKIs) 
such as Pazopanib is now first line therapy, but their effect on VHL-associated 
CNS HBs remains unknown. We report the use of Pazopanib in a patient with VHL 
disease for treatment of RCC who also harbored multiple CNS HBs. Following 
initiation of treatment, a large cervical and a lumbar spinal HB regressed in 
size while the remaining CNS HBs exhibited stable or progressive disease. These 
findings highlight the multiplicity of factors contributing to hemangioblastoma 
development, even among tumors with a common germline mutation, and the 
potential limitations of TKIs, but additionally this report supports the 
conservative management of asymptomatic VHL patients with spinal HBs whereby 
tumor response to TKI treatment may alleviate or postpone the need for surgery.