To our knowledge, this presents the first reported case of a complete destruction of the dens, as a result of a purulent bacterial infection leading to atlantoaxial luxation. Our case demonstrates that infections of the atlantoaxial region are difficult to diagnose and are seen mostly in immunocompromised patients, such as patients with diabetes. Furthermore, it proves that other infections besides tuberculosis can lead to complete osteolytic destruction of the atlantoaxial joint.