Chylothorax refers to the accumulation of chyle in the pleural cavity. Although rare, it is the most frequent cause of pleural effusion in the neonatal period. Its incidence is estimated at one in 15,000 neonates. The causes of chylothorax are multiple, and there are several major types of chylothorax defined by origin, ie, traumatic (and iatrogenic) chylothorax, medical (spontaneous) chylothorax, and congenital chylothorax. A case of neonatal chylothorax following surgery for esophageal atresia and our therapeutic approach to this entity are presented. Conservative therapy with total enteral nutrition and drainage were sufficient. Treatment for chylothorax is essentially medical, ie, pleural drainage, removal of dietary fats, treatment of any medical cause, and use of drugs to reduce production of chyle. In the event of failure or reappearance of a large effusion, surgical treatment is needed.