Aim: The authors present the therapeutic management of a 12-year-old boy with known hereditary multiple exostosis syndrome (HME), who developed spinal cord compression symptoms caused by an exostosis of the C2 lamina. A perinatal brain lesion with tetraparesis delayed the recognition of the spinal cord compression substantially, which resulted in an extensive spur-like growth of the exostosis. Method: In comparison with already published cases, this growth pattern was rather unique and required consideration on the best surgical management. We decided to monitor the spinal cord function from positioning of the patient to skin closure and to modify the surgical steps of the laminectomy with initial lateral cutting of both hemilami-nae. Results: Electrophysiological monitoring helped to avoid spinal cord compression by inadequate head anteflexion during positioning. Lateral cutting of the hemilaminae C2 resulted in spontaneous extrusion of the exostosis with immediate improvement of the electrophysiological findings. The boy experienced a prompt improvement of his neurological deficits. Conclusion: The good surgical and clinical result confirm the value of the applied management concept.