Basilar invagination in a Chiari malformation associated with osteogenesis imperfecta in the pediatric population is a rare entity. We report a case of a seven-year-old female who presented with sudden-onset bilateral spastic quadriplegia and evidence of a basilar invagination on MRI. She underwent emergency decompression of the impinging odontoid via transoral approach followed by posterior wiring and fusion of the C1 and C2 vertebrae. Imaging modalities such as dynamic CT and MRI play a major role in delineating any craniovertebral anomalies and neural impingement not easily identified in plain radiographs. Understanding the complex craniovertebral junction (CVJ) anatomy and the possible causes of such deformities is vital for ensuring proper diagnosis and management of these patients.
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