The authors report on the case of a 20-year-old man who presented with a transient tetraparesis. Neuroimaging studies demonstrated atlantoaxial dislocation and ventral compression of the rostral spinal cord caused by a quite rare association of os odontoideum and hypertrophic ossiculum terminale. The patient underwent removal of two free ossicula via a transoral approach and posterior fusion in which an autogenous bone graft was placed. The majority of cases of os odontoideum are believed to be an acquired form; however, controversy with regard to the congenital causes of os odontoideum remains. One hypothesis is that os odontoideum results from the failure of fusion and the hypertrophy of the proatlas, although considerable confusion surrounds this hypothesis because definitive classification of os odontoideum-to differentiate between similar anomalies-has not been established. This rare coincidence in the current case supports the belief that os odontoideum has a different embryological origin from ossiculum terminale, which is thought to be a proatlantal remnant.