This is the joint most frequent complication (with retinal angiomatosis) of VHL disease. Approximately 30 % of cerebellar hemangioblastomas occur as part of VHL disease, and evidence of VHL disease should be sought in all patients with apparently sporadic cerebellar hemangioblastoma. Tumours complicating VHL disease occur, on average, at a younger age than sporadic cerebellar hemangioblastomas, and in about 20 % of cases, the Tumours are multiple or recurrent. CT scanning demonstrates a contrast- enhancing mass, but MRI scanning is more sensitive and is preferred – particularly for surveillance. These Tumours are benign, may be cystic or solid, and the histological appearance is identical to that of a retinal angioma (hemangioblastoma). Approximately 4 % of patients with apparently sporadic cerebellar hemangioblastoma have a germline VHL gene mutation (Hes et al. 2000). In view of the risk of false-negative mutation analysis and somatic mosaicism, apparently sporadic early-onset cases may be kept under review in case evidence of VHL disease develops later. Surgery is usually performed when a hemangioblastoma becomes symptomatic. Stereotaxic radiotherapy may be occasionally an option for small non-cystic hemangioblastomas that are not amenable to standard surgery (Patrice et al. 1996).