We report here a rare case of high cervical stenosis at the level of atlas who presented with progressively deteriorating quadriparesis and respiratory distress. A 10-year-old boy presented with above symptoms of one-year duration with a preceding history of trivial trauma prior to onset of such symptoms. Cervical spine MRI revealed a significant stenosis at the level of atlas from the posterior side with a syrinx extending above and below. High-resolution computed tomography of the above level yielded an ill-defined osseous bar compressing the canal at the level of C(1) posterior arch, which appeared bifid in the midline. The patient was immediately taken up for surgery in view of his respiratory complaints. The child showed an excellent recovery after excision of the posterior arch of atlas and removal of the compressing osseous structure.