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Background: Mucopolysaccharidosis (MPS) is a multisystemic storage disorder of glycosaminoglycan deposits. Infiltration of the dura mater and supporting ligaments caused spinal cord compression and consecutive myelopathy, especially at the cranio-cervical junction (CCJ). Craniocervical instability and posterior decompression often raise the problem of fixation in children. The main purpose of this paper was to report the result of an original technique of occipito-cervical arthrodesis using a cranial halo-cast system in pediatric population. Methods: We recorded 21 patients with cervical myelopathy. All of them had spinal cord decompression by enlargement of the foramen magnum, C1 laminectomy, and occipito-C2 fusion using corticocancellous bone graft. Only one child has an extended laminectomy from C1 to C3. The occiput-C2 arthrodesis was stabilized by the cranial halo-cast system. This immobilization was performed preoperatively and kept for three months then switched to rigid cervical collar. Clinical assessment, including the Goel grade and mJOA, radiographs and magnetic resonance imaging were performed before surgery. The occipito-cervical arthrodesis was controlled by standard X-rays and CT scan. Results: According to the type of mucopolysaccharidosis, the patients were divided into MPS type I: n= 3, II: n=7, IV: n=11. The mean age of patients at surgery was 6.76 years. All mucopolysaccharidoses cases required a foramen magnum decompression by craniectomy, C1 laminectomy and occipito-C2 arthrodesis. As major complications, a child had immediate post-operative paraplegia due to spinal cord ischemia. The postoperative follow-up ranged from 1.5 to 4 years, with an average of 3.3 years. The average preoperative mJOA score was 8.9, and it improved to 14 points at the last follow-up. Conclusions: Satisfactory fusion and good clinical results were obtained with the 2-stage approach to CCJ anomalies.