Abstract:Gorham-Stout disease (GSD) is a sort of idiopathic osteolysis disease, in which with involvement of spine is rare. The diagnose and treatment of GSD are particular challenging as the pathological mechanism is currently unclear and the course of disease is variable. When the osteolysis has resulted in pathological fracture or deformity, early surgical intervention is able to facilitate new bone formation and arrest the progression of osteolysis. In this report, the patient who was characterized by multiple osteolysis of lumbar vertebrae and spinal deformity, was treated by medication and three-time surgical interventions. During the last surgery, the soft tissues where adjacent to the damaged vertebrae were infiltrative destructed. The massive hemorrhage had forced us to simplify the surgical procedure. With a 26-month follow-up, we observed that the osteolysis and the deformity were halted. The progression potential is high in the GSD with involvement of multiple vertebrae. Even the deformity is mild, one-stage long-segmental fixation and fusion remains a reliable choice.