This chapter discusses the pathology and management of this rare tumor. Hemangioblastomas are benign, solid or cystic, slowly growing, highly vascular lesions of uncertain histogenesis. Most hemangioblastomas are sporadic and associated VHL disease in approximately 25% of cases. The VHL disease is dominantly inherited and associated with multisystem, familial cancer syndrome predisposing to tumors of the eyes, CNS, kidneys, and other organs. Most commonly seen in posterior fossa (7-12% of posterior fossa tumors). The CT and MRI reveal nodular lesion often with peritumoral cyst. Polycythemia is associated with intracranial ones (not in spinal tumors) and may be used as tumor marker. No piecemeal excision of the mural nodule; must be treated as AVM. Radiosurgery may be an option. No need to excise the cyst wall as in cystic glioma. Radiotherapy in multiple or in deep seated lesions.