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Chordomas are histologically benign tumors with local aggressive behavior. They arise from embryological remnants of the notochord at the clivus, mobile spine, and sacrum. Chordomas are rare tumors in the pediatric age group. Their surgical management is difficult, given their propensity for inaccessible anatomical regions, and proximity to critical neurovascular structures. While en bloc resection with surgical margins has been advocated as the preferred approach for chordomas, tumor characteristics and violation of adjacent anatomical boundaries may not allow for safe en bloc resection of the tumor. Here, the authors present the case of a C1 chordoma in a 5-year-old boy with epidural and prevertebral extension. The patient’s treatment consisted of a far-lateral approach for resection of the tumor and C1 arch, followed by circumferential reconstruction of the craniocervical junction with an expandable cage spanning the skull base to C2, and posterior occipitocervical spinal instrumentation. At 42 months after surgery, the patient remains neurologically intact with stable oncological status, and no evidence of craniocervical junction instrumentation failure.