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Background Gorham-Stout syndrome (GSS) with spinal involvement is extremely rare, and bilateral chylothorax as a complication is usually fatal. In our case, pleural effusion appeared in the left hemithorax after ligating the right thoracic duct. Case presentation A 14-year-old patient presented with GSS affecting the thoracic spine with bilateral chylothorax. The case was successfully managed using...