Os odontoideum (OO) is defined as an ossicle with circumferential smooth margins and no osseous continuity with the body of axis. While the size of the ossicle is variable, it has a smooth surface, therefore, the cause of this condition has been presumed to be both congenital and traumatic. The possible cause of this condition has been implicated in its underlying etiology. However, patients with this condition may be asymptomatic or may be symptomatic of a variety of neurological deficits and vascular dysfunctions. The treatment of both the asymptomatic and symptomatic characteristics of this condition has undergone changes over the past few decades. The most challenging issue encountered is the management of OO with irreducible atlantoaxial dislocation. This outlines the objective of our discussion to examine the aforementioned subject extensively and review the appropriate potential strategies for the treatment of this condition.