Tehran University of Medical Sciences Journal of Orthopedic and Spine Trauma 2538-2330 11 3 2025 09 03 124 8 Maziar Malekzadeh Department of spine surgery, Tehran University of Medical Sciences, Tehran, Iran Babak Mrzashahi Department of Spine Surgery Imam Khomeini Hospital Complex, Tehran University of Medical Sciences, Tehran, Iran Siamak Shabani Department of Spine Surgery Imam Khomeini Hospital Complex, Tehran University of Medical Sciences, Tehran, Iran 2025 04 07 2025 09 03 Background: Congenital factor VII (FVII) deficiency is a rare autosomal recessive coagulation disorder that poses significant challenges during major surgeries, particularly high-risk procedures like instrumented spinal fusion for adolescent idiopathic scoliosis (AIS). Case Report: We present the case of a 15-year-old girl diagnosed with Lenke type 2 AIS and severe congenital FVII deficiency. She was referred to Imam Khomeini Hospital Complex, Tehran, Iran, for corrective spinal fusion. Preoperatively, a single dose of recombinant activated FVII (rFVIIa) at 20 mcg/kg was administered to maintain hemostasis. The instrumented spinal fusion was performed under general anesthesia using meticulous surgical techniques. Notably, no additional doses of rFVIIa were required during the procedure. Conclusion: This case demonstrates that with appropriate preoperative factor replacement and careful intraoperative hemostatic management, patients with severe FVII deficiency can safely undergo corrective spinal fusion. It underscores the critical importance of sustaining hemostasis in managing high-risk surgical patients with coagulopathies. https://jost.tums.ac.ir/index.php/jost/article/view/669 https://jost.tums.ac.ir/index.php/jost/article/download/669/429