Gorham's disease is an extremely rare bone disorder that manifests as spontaneous osteolysis with abnormal blood or lymphatic vessel growth. Chylothorax is not uncommon, but it can be fatal in patients with Gorham's disease. Herein, we report a 4-year-old boy with Gorham's disease who presented with bilateral chylothorax and spontaneous osteolysis extending from the lower thoracic spine to the sacrum with involvement of the right iliac bone. The condition was successfully treated by thoracoscopic surgery combined with mechanical and chemical pleurodesis. There was no evidence of recurrence of chylous pleural effusion at 3-year follow-up. His progressive osteolysis has been controlled by subcutaneous injection of interferon alpha-2b for the past 2 years.