Introduction: Occipito-condylar hyperplasia is a very rare anomaly of the cranio-vertebral junction that was only reported in two patients before and managed through posterior approach.
Case material: A 10-year-old girl with a sudden attack of quadriparesis and respiratory distress was admitted to our center. A detailed work up favored a high cervical myelopathy due to bilateral occipito-condylar hyperplasia and Chiari malformation.
Results: An endoscopic endo-nasal approach under navigation guide was used to drill the compressive lesion.
Conclusion: Our patient is added to the literature as the third one that was approached through a different surgical corridor. Successful decompression with excellent results was gained.