In a retrospective review of the cases of thirteen skeletally immature children and adolescents (four to eighteen years old) with instability of the upper part of the cervical spine (occiput to fifth cervical vertebra), we determined the efficacy of posterior arthrodesis and halo-cast immobilization in the management of this condition. The patients were divided into two groups: those with congenital vertebral anomalies alone (fusion or structural defects, or both) and those with cervical anomalies and systemic disorders (dwarfism, juvenile rheumatoid arthritis, Down syndrome, and cerebral palsy). Two patterns of instability were found: instabilities at intervertebral joints adjacent to vertebral fusions, and instabilities located in vertebral defects. For all patients treatment included a posterior arthrodesis with external immobilization by a halo cast, and in two patients internal fixation with wire was also used. Solid arthrodesis was obtained in the twelve patients who were treated with autogenous grafts (iliac cancellous bone in eleven and rib bone in one), and a non-union developed in a child who was treated with bank-bone rib segments. Posterior cervical arthrodesis with wire fixation carries some risk of neural injury and often is not applicable in children with anomalous vertebrae. Spine fusion using delicate exposure, decortication using an air-drill, and placement of autogenous cancellous iliac grafts with external immobilization by a halo cast minimizes the risk of neural damage and is a reliable way to obtain a solid arthrodesis.