A case is presented with severe infra- and supratentorial subarachnoid haemorrhage (SAH) caused by intramedullary haemangioblastomas of the cervical spinal cord. The patient initially had a typical SAH symptomatology without neurological deficit. The cerebral angiogram was nondiagnostic. After admission the patient developed slightly progressive right sensorimotor paresis. Angiography of the cervical spine and MRI delineated three intramedullary haemangioblastomas. Retrospectively the diagnosis of von Hippel-Lindau (VHL) disease was made by multiple haemangioblastomas and a positive family history. The three lesions were surgically completely removed.