Objective: Paediatric scoliosis (PS) and Chiari malformation type 1 (CM-1) have been reported to be associated with each other. Scoliosis curvature is a common finding among patients operated for CM-1, and curve development has been related to it. We report a cohort of PS and CM-1 patients managed with posterior fossa and upper cervical decompression (PFUCD) by a single surgeon, with an average of two years of follow-up. Methods: We present a retrospective cohort in a single referral centre for patients with CM-1 and PS. Results: From 2011 to 2018, we identified fifteen patients with CM-1 and PS; eleven underwent PFUCD, ten had symptomatic CM-1, and one had asymptomatic CM-1 but showed curve progression. The remaining four CM-1 patients were asymptomatic and were hence treated conservatively. The average follow-up post-PFUCD was 26.2 months. Scoliosis surgery was performed in seven cases; six patients underwent PFUCD prior to the scoliosis correction. One scoliosis case underwent surgery in the presence of mild CM-1 treated conservatively. The remaining four cases were scheduled for scoliosis correction surgery, while three were managed conservatively, with one case lost to follow-up. The average time between PFUCD and scoliosis surgery was 11 months. None of the cases had intraoperative neuromonitoring alerts or perioperative neurological complications. Conclusion: CM-1 with concomitant scoliosis can be found. Symptomatic CM-1 might require surgery, but as we discovered, PFUCD had negligible effect on curve progression and the future need for scoliosis surgery.