A 39-year-old man with a history of von Hippel-Lindau (VHL) syndrome presented with acute onset of headache at our emergency department. He previously underwent multiple neurosurgical resections of cerebellar and spinal hemangioblastomas. During his most recent surgery, which was roughly 1 year ago, a symptomatic right-sided large and cystic cerebellar hemangioblastoma was resected. The most recent radiological follow-up (2 months before presentation) showed multiple stable asymptomatic hemangioblastomas. Aside from the headache, the patient reported no other symptoms nor a history of head trauma. Neurological examination and coagulation assessments were normal.