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Many excellent reports have dealt with the various aspects of cranial chordoma. It remains a relatively rare neoplasm, particularly in younger children. The authors have had the opportunity to treat a 5-year-old child harboring a basiocciput chordoma. It extended from the mid-clivus to C3. A transoral labiomandibular approach was used, allowing its resection. No evidence of recurrence was noted 3 years later. A literature search confirmed the rarity of basiocciput chordoma in young children. The perioperative difficulties encountered prompted this report.

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