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Gorham–Stout disease (GSD) is characterized by aggressive bone resorption, proliferation of vascular or lymphatic vessels, and soft-tissue swelling. Bones that initially appear normal start to resorb, partially or completely. However, the etiology of GSD is unknown.A 29-year-old man with a chief complaint of toothache and mobility in the lower right mandible for the previous 1 year.Gorham–Stout disease (GSD).The RANK-ligand inhibitor denosumab was suggested to use to inhibit the development of osteoclasts and slow mandibular resorption. In addition, we proposed resection of the remaining mandible and reconstruction via vascularized bone graft, after resorption of the mandible had become stationary.Regular follow-ups were advised to this patient to monitor the stability of bone resorption prior to any surgical intervention.We strongly recommend that every attempt should be made for early diagnosis and prompt effective medical and surgical management. The failure to do so results in further complications and poor prognosis.