Two cases of a rare entity, spinal extrathecal hemangiolipoma, are presented. The clinical and histological features of the tumor are discussed, with particular attention to the uncommonly sudden onset of symptoms in one patient. Complete neurological recovery was exhibited by one patient after excision of the lesion, whereas only a partial recovery was achieved in the other case. The pathogenetic aspects of hemangiolipomas and the literature dealing with the previously reported 12 cases are reviewed.