BackgroundOsteoradionecrosis (ORN) of the upper cervical spine is a rare but severe complication of head and neck cancer radiotherapy. To raise awareness of this condition, we describe a patient with a history of nasopharyngeal carcinoma who developed ORN of the upper cervical spine and review the published literature reporting surgical management.Case presentationA 59-year-old female patient with persistent neck pain for one month and limited range of neck motion who had undergone radiotherapy for nasopharyngeal carcinoma with a total dose of 69.96 Gy 15 years ago presented to our hospital. The patient underwent endoscopic transnasal and transoral resection of the odontoid process and C1 anterior arch, combined with occipitocervical fusion. To better understand surgical management of ORN of the upper cervical spine, the literature published in the PubMed, Ovid MEDLINE, and Embase databases was reviewed. Our patient experienced alleviation of cervical pain and did not exhibit any postoperative complications. Since 2005, 11 cases of surgical management of ORN of the upper cervical spine (including the present case) have been published. Basilar invagination and/or atlantoaxial subluxation were observed in 4 /11 cases. Endoscopic procedures were performed in 4/11 cases, and occipitocervical fusion was performed in 8 /11 cases.ConclusionEndoscopic transnasal and transoral resection of the odontoid process and C1 anterior arch is a safe and effective treatment option for ORN of the upper cervical spine. Occipitocervical fusion is useful in patients with basilar invagination and atlantoaxial subluxation.