The clinical differences were evaluated in spinal hemangioblastoma between patients with sporadic disease and patients with von Hippel-Lindau (VHL) syndrome. The distribution of hemangioblastomas in the central nervous system was investigated in 56 patients treated between 1988 and 2008 at the University of Tokyo Hospital. The characteristics of spinal hemangioblastomas were compared in 35 patients including 17 with sporadic disease and 18 with VHL syndrome treated between 1988 and 2008 at our hospital and three affiliated institutions. Spinal hemangioblastomas were much more prevalent in patients with VHL syndrome (88.2%) than in patients with sporadic disease (20.5%, p < 0.001). Spinal hemangioblastomas associated with VHL syndrome were diagnosed a decade earlier (p = 0.007) and were associated with less severe neurological symptoms than sporadic lesions (p = 0.004). Most patients with sporadic disease had a single lesion at the cervical or thoracic level, whereas patients with VHL syndrome had multiple lesions at all spinal levels (p = 0.04). Patients with sporadic disease exhibited significant improvement in postoperative neurological status (p = 0.02), whereas patients with VHL syndrome had no change in status (p = 1.00). Number of removed lesions (p = 0.03) and lower spinal cord lesions (p = 0.05) were associated with poor surgical outcome. Tumor recurrence was correlated with partial removal of lesions (p = 0.05). One third of patients with VHL syndrome developed new lesions every 2 years. The major finding of this study is that the incidence of spinal hemangioblastoma, distributed through all spinal levels, may be as high as 88% in patients with VHL syndrome, which is much greater than previously reported.