Aim:The craniovertebral junction is a remarkable anatomical area with unique anatomical and functional relationships, which present difficult challenges for surgeons. The traditional approach to addressing pathologies in this area is microscopic transoral resection with posterior fixation; however, endoscopic endonasal clivus and odontoid surgeries now currently gaining interest as alternative approaches. We reviewed our experience of using the endoscopic endonasal approach at our institution.Material and Methods:We retrospectively evaluated 41 patients (21 male, 20 female; age range, 2–65 years) who underwent endoscopic endonasal procedures for craniovertebral junction pathologies between 2008 and 2017.Results:Of the 41 patients, 27 had clivus lesions, 7 had odontoid lesions, 6 had basilar invagination and 1 had rhinorrhea repair. Six patients underwent an additional posterior decompression/fusion either before or after the endonasal procedure. None of the patients required tracheostomy and cerebrospinal fluid leakage was detected in one patient postoperatively. The patients’ mean modified Rankin scale and visual analog scale scores were 3 and 4 respectively. The follow-up period ranged from 12 to 50 months.Conclusion:Although the microscopic transoral approach has been considered the gold standard for craniovertebral junction surgical management, endoscopic approaches are feasible, safe, and effective for addressing pathologies in this region, with developing technique and experience.