Abstract Introduction: Subarachnoid hemorrhage (SAH) secondary to spinal hemangioblastoma (HBL) is extremely rare, with only a few case reports to date. We report the experience of our reference center for spinal tumors and Von Hippel-Lindau (VHL) disease in patients with spinal HBL presenting with SAH. We further performed a systematic review of the literature. Methods: We report two cases. A systematic search was performed using the PubMed, Embase and Cochrane databases, with no limit for publication date. Inclusion criteria were: patients with HBL presenting with SAH, with or without VHL. The systematic review retrieved only 10 studies, including 16 patients. Results: In our center, the first case concerned radicular HBL at D12 level, presenting with spinal and brain SAH. The patient underwent uneventful microsurgical en bloc resection. Postoperative course was normal. The second case concerned HBL with SAH at the cervico-medullary junction, with rapidly fatal course. The systematic review revealed female predominance, at a median age of 40 years, with HBL predominantly located at cervical level, common preoperative symptoms being headache and signs of meningeal irritation. Conclusions: In conclusion, spinal HBL is an extremely rare cause of SAH. The systematic review found putative risk factors: female gender, age 40-50 years, cervical location, and median size 2 cm. Diagnosis can be difficult when presentation mimics intracerebral SAH. We advocate early surgical removal. The risk of rapidly fatal course in case of major hemorrhage needs to be borne in mind.