Gorham-Stout disease is a rare skeletal disease which is progressive and spontaneous and idiopathic resorption of whole body bone. In the initial stage, the osteolytic site is replaced by histologically benign vascular proliferation without new bone formation and finally by dense fibrous tissues. We encountered with a patient involving the mandible with attention by the clinical, radiographic, scintigraphic, angiographic, and computed tomography. We resected the mandibular lesion which was reconstructed with a fibular free flap. We report literature review with a mandibular lesion involved with Gorham-Stout disease.