Langer-Giedion syndrome results from a genetic deletion on chromosome 8. Although redundant skin is part of Langer-Giedion syndrome, lower eyelid entropion and eyelid laxity have not been previously reported in the medical literature. We report a case of bilateral lower eyelid entropion in a patient with Langer-Giedion syndrome who successfully underwent entropion repair with anterior lamellar resection, lower eyelid retractor advancement, and lateral canthopexy. Although she has done well 6 months after surgery, we anticipate that laxity and eyelid instability will continue to develop with age, and additional reconstructive surgery may be required.