Os-odontoideum is a rare condition described radiographically and clinically as a congenital anomaly of the second cervical vertebra (axis). It is a smooth, independent ossicle of variable size and shape separated from the base of a shortened odontoid process by an obvious gap, with no osseous connection to the body of C2. This study reviewed the literature on OO to evaluate its etiology, clinical presentations, differential diagnosis, imaging modalities, and outcomes in the management of asymptomatic and symptomatic cases of Os Odontoideum. Key articles from PubMed, EMBASE, Google Scholar, and Cochrane were searched. Considering etiology, the traumatic hypothesis is favoured over the congenital hypothesis as per recent literature on OO. Clinical presentation varies from asymptomatic to mild neck pain to severe myelopathy and neurodeficit. Various C1-C2 instrumentation and fusion techniques like wiring, trans articular screw and laminar screws have been described with success rates. Os odontoideum is a rare condition with limited existing literature. Considering the significant risks involved if conservative management opts, like severe neuro deficit to sudden death on trivial trauma and the recent improvement of imaging tools helping to understand the pathology of the disease, surgery can be indicated even in an incidentally detected os odontoideum. However, a case-by-case approach can be considered for stable asymptomatic patients depending on factors such as age, activity level, comorbidities, syndromic association, and radiographic findings.