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Background Hemangioblastomas (HBs) are rare lesions accounting for 1%–5% of all spinal cord tumors, and are mostly associated with Von Hippel-Lindau (VHL) syndrome. Localization in the cauda equina is uncommon. Aim: In this manuscript, we aimed to describe a rare case of sporadic intradural extramedullary HB of the cauda equina and present a literature review. Mathods: A systematic research was performed on PubMed, MEDLINE, and Google Scholar, using the keywords “spinal HB” and “cauda equina tumors.” The previous literature is integrated by the description of the present case. A 49-year-old female presented in August 2020 to our institution with a magnetic resonance imaging (MRI) which showed an intradural mass at L1/2 level and angiography that showing a nidus of serpiginous vessels inside the lesion. Symptoms were right sciatica and paresthesia in right L5 radicular dermatome for more than 3 months. Neurological examination revealed claudicatio spinalis and hypoesthesia on right L5 dermatome and weakness of right anterior tibialis muscle. Microsurgical en bloc resection of lesion was performed with adjuvant neurophysiological intraoperative monitoring. The histological examination provided the diagnosis of HB. Results: After surgery, symptoms and neurological impairment gradually improved. Postoperative MRI showed no residual tumor. Conclusions: Although intradural extramedullary HB of the cauda equina without VHL syndrome is a rare pathological entity, this diagnosis must be taken in consideration when a mass affects cauda equina. Preoperative embolization is an option to minimize intraoperative bleeding. Radiosurgery seems to prevent recurrences when the tumor is not completely excised. A complete surgical removal of the lesion is usually possible and it leads to a low likelihood of recurrence.