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Congenital limb deficiency requiring transfemoral amputation. A healthy female infant was born from a twin pregnancy with an isolated congenital lower extremity malformation. Aside from prenatally diagnosed polyhydramnios, the infant had normal prenatal and postnatal diagnostic workup. She underwent transfemoral amputation and healed uneventfully. Congenital limb anomalies may be the result of an unidentified amniotic band, thromboembolic event or twin-twin transfusion syndrome, though in this case, prenatal screening did not indicate any evidence of a limb anomaly and postnatal workup was negative.