We present the case of a 26-year-old girl with pleural effusion and destruction of adjacent ribs and vertebrae. An open biopsy revealed the typical histopathological changes of Gorham's disease. Pleural effusion was treated with drainage, pleurodesis and high dose radiation therapy. The disease process was arrested and she remains well with no sign of recurrence at an 18-month follow up. Now we are observing spinal deformity and considering early surgical stabilization.