Pleural diseases are rare in the neonatal period, but sometimes are associated with significant morbidity and mortality. Congenital chylothorax is the most common type of pleural effusion in neonates. The diagnostic approach to neonatal chylothorax and neonatal pleural effusions are discussed in detail. The management of congenital chylothorax is challenging, because it includes prenatal procedures, diet, drug treatment, and surgery. Summarized treatment protocol gives information about the most important therapeutic measures, according to the postnatal age and clinical evolution. Brief description of the other types of pleural effusions is provided.