Chylothorax is the accumulation of chyle in the pleural space. In newborns the congenital form is often prenatal diagnosed, while the late variety originates to damage to the thoracic duct by cardiac surgery, diaphragmatic hernia, etc. Clinical presentation results from the accumulation of pleural fluid and the symptoms depends on the size of the effusion. The treatment needs both medical and surgical care. The pleural cavity should be drained via thoracocentesis, and total parenteral nutrition should be started. Afterward fat-free diet with the addition of medium-chain triglycerides could be initiate. Somatostatin and octreotide have been successfully employed, mainly in post-surgery chylothorax. Surgery should be considered when medical management fails. Some approaches are reported, and thoracic duct ligation, pleurodesis and pleuroperitoneal shunts are the most utilized. The prognosis of chylothorax depends on the etiology, and it is consequence of a variety of treatments that may influence the outcome.