Gorham disease is an extremely rare condition of unknown etiology characterized by progressive osteolysis. Only 28 cases of its spinal involvement have been reported, and some of those cases showed kyphosis, kyphoscoliosis, subluxation or dislocation. No definite regimen of treatment has been established yet. A 10-year-old boy presented with a severe and progressive kyphosis over 90 degrees caused by Gorham disease from T3 to T12. In situ posterior fusion with a hook and rod system and iliac bone grafts were performed, but after surgery, he had complete paraplegia and its cause was uncertain. Based on the unfortunate consequence of the present case and the review of the literature, we propose the treatment strategies for spinal Gorham disease.