Os odontoideum is a rare anomaly of the odontoid process. When unstable, it can be a source of neck pain that can result in serious neurological injury. In young children, the presence of a transverse lucency at the base of the dens represents the normal developing growth plate. However, os odontoideum should be considered in the differential diagnosis in a child with recurrent or persistent neck pain. A case of an unstable os odontoideum in a two-year-old child is presented to illustrate this point.
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