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Background: Chordomas are primary bone tumors derived from the embryonic notochord. They represent 1-4% of all malignant bone tumors. They have a predominantly extra-axial location, arising in the clival region in 35% of reported cases. The prognosis is generally poor, and radical resection remains the first-line treatment. This study aims to describe a case of a clival chordoma that was resected through a combined microscopic transoral and endoscopic endonasal approach, with excellent clinical outcomes., Case Description: A 24-year-old woman with low cranial nerve symptomatology was admitted for a two-stage surgical approach. An occipital-cervical fixation was performed in the first stage, while a combined endonasaltransoral resection was performed later for tumor resection., Conclusion: Microscopic transoral and endoscopic endonasal approaches offer advantages for treating clival chordomas, with careful consideration of anatomical constraints and potential for postoperative recurrence being essential in approach selection., Competing Interests: There are no conflicts of interest., (Copyright: © 2024 Surgical Neurology International.)