Congenital femoral deficiency (CFD) with primary distal involvement (Paley Type 4) is the rarest phenotype in a spectrum that affects roughly 1 in 40,000-100,000 live births. Such children present early with pronounced limb length discrepancy (LLD), genu valgum, and patellofemoral maltracking. We describe a 4-year-old girl who exhibited an 8 cm right-sided LLD, severe distal femoral valgus, and hypoplasia of the lateral femoral condyle. A four-ring Ilizarov frame was applied, and two corticotomies were performed in a single sitting: (1) an oblique distal osteotomy to open-wedge correct valgus while permitting 1 mm day distraction and (2) a midshaft transverse osteotomy lengthened at 1 mm day. After 5 months, 12 cm of regenerated bone restored mechanical alignment and equalized limb length. The patient achieved full, painless knee and hip motion and resumed unrestricted play 7 months after frame removal. No deep infection, neurovascular injury, knee contracture, or regenerate fracture occurred. The case illustrates that carefully planned, simultaneous deformity correction and lengthening can safely deliver length gains beyond those typically reported in single-level procedures while obviating multiple staged surgeries.
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