Background: Gaucher disease is a rare hereditary glycolipid storage disease. One of the rare complications is neurodeficits due to vertebral involvement. Case presentation: An 18-year-old female patient presented to the outpatient clinic with cauda equina syndrome due to sacral involvement of type 1 GD. Bilateral laminectomy via posterior approach without posterior stabilization was performed. Conclusion: Maximum excision of the mass avoiding destabilization of the spinal column can provide long-term vertebral stability and improvement in neurodeficits.