[A rare differential diagnosis of chylothorax].

We present the case report of a 17-year-old patient with right pleural effusion and pre-existing lymphangiomas of the chest wall. Thoracentesis revealed a salmon-milky effusion containing chylomicrones defining a chylothorax (with blood intermixture). A CT scan and MRI showed multiple osteolysis. After excluding malignancy as well as infectious and traumatic causes of the chylothorax, we made the diagnosis of a Gorham-Stout syndrome. A minimal-invasive pleurectomy and decortication were done successfully and prevented a relapse of the pleural effusion for at least one year.