Study Design Retrospective study. Objective To assess results of posterior instrumented fusion using pedicle screws in 12 children with osteogenesis imperfecta (OI) with spinal deformity at a single institution from 2001 to 2012. Summary of Background Data This is the first case series of OI patients who underwent non–cement augmented screw-rod instrumented fusion published in the literature. Methods Of a total of 54 children with spinal deformity associated with OI, 12 (22.2%) were submitted to posterior spinal fusion with pedicle screws (80% density) because of severe spinal deformity. Here we reported the results in seven females and five males. Results Five thoracic (41.7%), five double (thoracic and lumbar 41.7%), and two lumbar (16.7%) curves were considered. The mean number of fused levels was 11.8 (range: 5 to 16). Mean age at surgery was 13 years 8 months. Mean follow-up was 7 years 11 months (range: 3 years 7 months to 16 years 1 month). The mean preoperative scoliosis angle was 75.6°, whereas the postoperative angle was 31.4° (58.5% correction rate). The mean preoperative kyphosis angle was 57.4° and the postoperative angle was 42.3°. We observed one superficial infection, one dural tear, and three cases of proximal junctional kyphosis; two patients required one revision surgery each (2 years and 4 months postoperatively on average). Conclusions To our knowledge, this is the first case series published in the literature regarding OI with instrumented fusion with non–cement augmented pedicle screws exclusively in children with spinal deformity. We found that posterior spinal fusion with the screw-rod system in OI deformity in children is feasible and reliable, and has acceptable clinical and imaging results in the long-term follow-up. Level of Evidence Level IV © 2017 Scoliosis Research Society