Gorham-Stout disease (GSD) is a rare progressive osteolytic disease with intraosseous lymphangiomatosis. A previously healthy 55-year-old woman presented with left leg hypoesthesia and upper back pain. k pain, back pain, and bilateral shoulder pain. In radiologic examination, there was an osteolytic lesion in T3, 4, 5, 6 vertebra. And a dislocation in the T3/4 vertebrae and a compression fracture in the T4 vertebra were founded. She was treated with posterolateral fusion (PLF) from C7 to T7. For 1 year, she had no specific symptoms but a new osteolytic lesion was found on thoracic T8 on 1 year follow-up magnetic resonance imaging. Radiotherapy was performed with 40 Gy, and chemotherapy was performed with denosumab. This report described a case of GSD that involved the thoracic spine and was appropriately managed. In the present case of a middle-aged Asian woman, correction of severe thoracic instability and prevention of neurologic compromise were accomplished through PLF from C7 to T7. Recurrence was observed 1 year later, but it was appropriately treated with neoadjuvant therapy.