Behçet's syndrome is a chronic multisystem inflammatory disease characterised classically by recurrent oral and genital ulcers with ocular lesions. It can affect blood vessels of all sizes, but involves veins more commonly than arterie. The presence of chylothorax in Behçet's syndrome is rare, with only a few cases cited in the literature. The most likely pathogenesis is SVC thrombosis with obstruction of the orifice of the thoracic duct resulting in leakage of chyle from the pleural lymphatics into the pleural space. The majority of the previously reported cases were managed medically without surgical intervention. We believe that this report describes the first use of surgery to ligate the thoracic duct and create a pericardial window in a Behçet's syndrome with chylothorax and chylopericardium.