Craniocervical junction chordomas (CCJC) are rare malignant tumors that invade the skull base and lead to bone destruction and instability. [1][2][3] En bloc removal with wide margins is the treatment of choice. 4 The extreme far-lateral approach provides control of the CCJ neurovascular structures and the tumor-brainstem interface. 5,6 A 30-year-old woman was diagnosed with CCJC for which she had undergone multiple surgical resections with adjuvant radio/immunotherapy. She presented to us with worsening bilateral paresthesia, lower and upper extremity weakness, and imbalance. Imaging showed extensive growth of the disease involving the upper cervical spine, from CCJ to C3, extending into C2-C3 foramina, encasing the vertebral artery (VA), and severely compressing CCJ and upper cervical cord. A 3-stage procedure was planned with the goal of complete tumor resection, and the patient consented to the procedure and to the publication of her image. On day 1, she underwent angiography for balloon test occlusion and coil sacrifice of the V4 segment of the involved nondominant VA. On day 2, an extreme lateral transcervical approach with odontoidectomy, C2-C3 corpectomy and laminectomy, right side C1 to C3 posterior rhizotomy, anterior arthrodesis with bone allograft, and near en bloc gross total resection was performed. On day 3, she underwent occipitocervical fusion. Postoperative course was uneventful with immediate improvement of symptoms and complete resolution at 6 weeks postoperatively. Early and 3-month postoperative imaging showed complete resection of the lesion, restoration of cervicomedullary and cervical cord configuration, well-placed instrumentation, and fusion construct, both anterior and posteriorly.