Introduction: Although uncommon, fractures of the os odontoideum are known to occur in children under 7 years old, following acute trauma.
Clinical picture: We report a case of chronic subluxation of the os odontoideum resulting in cervical myelopathy in a child with Beare-Stevenson cutis gyrata syndrome after surgery to the head and neck.
Treatment and outcome: The patient was initially put in a Halo vest, following which occipital cervical fusion was performed.
Conclusion: Subluxations and fractures at the odontoid synchondrosis are rare but should be anticipated in young children with risk factors for instability of the cervical spine.