The odontoid process is located in a critical area of the spine and is part of a complex structure, the atlanto-axial junction. This C1-2 junction allows for a great deal of cervical mobility in planes of rotation, flexion, and extension. Lesions of the odontoid process and associated ligaments can result in compression of the brainstem, cervicomedullary junction, and spinal cord. Resultant symptoms include weakness, spasticity, lower cranial nerve palsies, including difficulty in swallowing, phonation disorders, Bell's cruciate paralysis, and breathing disorders. Patients with lesions in this area require a thorough neurological and otolaryngologic evaluation. The reducibility of the lesion needs to be determined as an operative prerequisite. Extensive multimodality radiographic imaging may be required pre-, intra-, and postoperatively. Transoral surgery of the odontoid is technically demanding and requires a thorough understanding of the relevant anatomy and pathology. Perioperative and intraoperative adjuncts such as the operating microscope, fluoroscopy, image guidance instruments, high-speed air drills, and somatosensory-evoked monitoring help to expedite the surgery and reduce the risk of complications. Postoperative instability must be assessed and treated. A team consisting of a spine surgeon, anesthesiologists, otolaryngologists, and critical care physicians skilled in managing patients with similar conditions may provide the best possible outcome for these patients. This article reviews the pertinent patient evaluation, imaging studies, surgical indications and techniques, and post-operative management.