Abstract:[Objective] To introduce the surgical technique and preliminary clinical results of autologous rib structural bone grafting in instrumented fusion of craniocervical junction zone in children. [Methods] Two children received atlantoaxial, or occipitocervical instru- mented fusion with rib autografts for congenital craniocervical junction disorders. A posterior cervical median incision was made, and the at- lantoaxial pedicle screw-rod, or occipitocervical plate-screw-rod system was routinely placed for internal fixation. A high-speed burr was used to grind the bone graft groove at the posterior inferior edge of the atlantoaxial arch, or occipital and the superior edge of the cardinal ver- tebral plate. Then, an oblique incision was made along the direction of the ribs under the subscapular angle of the posterior chest wall to ex- pose the rib, a rib segment was harvested and trimmed into columns in proper length, and placed on the bone bed on the craniocervical junc- tion zone. [Results] The surgical procedure was successfully performed in both patients, without hemopneumothorax and postoperative com- plications in the rib donor site, and were followed up for 6 months and 12 months, respectively. Both patients got significantly symptom im- provement postoperatively in terms of JOA and VAS scores. Solid atlantoaxial or craniocervical fusion was noted with bony rib healing in the donor site 3 months postoperatively. [Conclusion] The autologous rib structural bone grafting is a safe and effective instrumented fusion of the craniocervical junction area in children.