Fetal pleural effusion can be an isolated or associated with fetal hydrops. The incidence of isolated pleural effusion is 1 in 10,000 to 1 in 15,000 pregnancies. Congenital Chylothorax can occur in isolation or more commonly in association with certain genetic syndromes such as Noonan’s syndrome, Turner’s syndrome and trisomy 21 lymphatic malformations anemia, cardiac defects, aneuploidy, viral infections and lung lesions. The effusion may increase in size causing caval compression, hydrops and fetal death. Swallowing of amniotic fluid may restrict the gut leading to polyhydramnios and ultimately lung compression leading to pulmonary hypoplasia. In 50% of cases, the symptom of Chylothorax is present at birth and develops during first week of life. In some cases, Congenital Chylothorax is associated with Turner Syndrome, Noonan Syndrome, Hydrops Fetalis, and Down’s Syndrome. Traumatic Chylothorax in newborn is iatrogenic after intrathoracic surgery. Cranial Ultrasonography and chromosomal assessment of fetus with pleural effusion, viral serology for intrauterine infection, Human Immunodeficiency Virus (HIV), parvoviruses and adeno viruses were investigated. Pleural tap for lymphocytes, triglycerites and proteins, sepsis screening were done. Asymptomatic infants with small pleural effusion resolve spontaneously. In symptomatic, treatment consists of drainage of chyle, supportive ventilation, supplementation of fluid loss, parenteral nutrition and treatment of sepsis if any. OK 432 in Ex-utero intrapartum (EXIT)) treatment with thoracocentesis, total parental nutrition and medium triglycerides (MCT), Fibrin glue with argon plasma fulguration & fibrin glue in a premature infant.