Von Hippel-Lindau (VHL) disease is a complex genetic syndrome characterized by multisystemic vascular neoplastic disorder. The affected population tends to develop tumors mainly involving the central nervous system, adrenal glands, pancreas, and kidneys. We describe anesthetic management for the resection of a cerebellar mass compressing the brainstem in a recently diagnosed 25-year-old female patient with a history of von Hippel-Lindau (VHL) syndrome. An uneventful occipital craniectomy for cerebellar tumor resection was performed under total intravenous anesthesia, without complications. The patient was discharged home on postoperative day five. This case depicts a situation in which a brainstem compressing lesion needs to be addressed urgently, and the pharmacological neuroprotective technique utilized for this procedure.