IntroductionChordomas are rare, locally aggressive tumors that often occur in the axial spine, especially in the sacrum. The treatment of chordomas located in the upper cervical spine is challenging. En bloc resection is the preferred surgical option for total tumor removal.Case presentationWe report the case of a C2 chordoma in a 47-year-old Thai woman. She was treated with a two-stage, anterior–posterior, C2 total spondylectomy with titanium mesh cage reconstruction and radiotherapy. The first stage involved posterior stabilization from the occiput to C5, a total laminectomy, and removal of the posterior rings of the bilateral foramen transversarium to preserve the bilateral vertebral arteries. The second stage comprised a transoral mandibular split with en bloc resection of C2, followed by titanium mesh cage reconstruction and kick-off anterior cervical plating. At the 5 year follow-up, no tumor recurrence was identified on magnetic resonance imaging. The patient had no neurological deficits but still had minor complications from the anterior transoral mandibular split.ConclusionsExcellent midterm results were obtained using a transoral mandibular split with reconstruction and posterior spinal fusion from the occiput to the lower cervical spine coupled with adjuvant radiotherapy. We recommend this approach as the treatment of choice for chordoma in the upper cervical spine.