Nonoperative management of fractures in the setting of diffuse idiopathic skeletal hyperostosis (DISH) or ankylosing spondylitis is often unsuccessful. The subaxial spine is a common site of hyperextension fractures in the setting of DISH. Fractures of the upper cervical spine are uncommon in DISH. We report, to our knowledge, the first case describing successful nonoperative management of a type 2 odontoid fracture in a patient with DISH. We discuss the patient’s initial presentation, physical examination, imaging findings, and management. A 73-year-old male presented with neck pain to the emergency department after sustaining a ground-level fall. Computed tomography of the cervical spine demonstrated a minimally displaced type 2 odontoid fracture in the setting of extensive DISH. He was immobilized with a hard cervical collar as the definitive management of his fracture. The collar was discontinued after 3 months. At his 2-year follow-up, he had a stable fibrous nonunion at the fracture site with tolerable neck pain. Flexion–extension radiographs demonstrated a stable alignment, and nonoperative management was continued. In selected patients with odontoid fractures in the setting of DISH, there is a role for nonoperative management alongside close monitoring.