Osteoid osteoma (OO) is a small tumor of bone that affects the spine in 10% of the cases. The tumor has a tendency to neural arc, and the lumbar spine is the most common site of presentation. Lesions of the odontoid process are very rare. We presented the case of a 20-year-old man who had cervical pain for 8 months. The pain responded to medical therapy. After investigation, there was a lytic lesion at the odontoid process with the characteristic features of OO in computed tomography scan and magnetic resonance imaging. Firstly, medical treatment was initiated with the administration of nonsteroidal anti-inflammatory drugs; however, due to adverse effects and worsening of his pain, the patient underwent surgical treatment with intralesional curettage from the transoral approach. In addition, the posterior fusion of the first and second cervical vertebrae was performed. The pain disappeared, and the patient was symptom-free after one year of follow-up.