Congenital chylothorax (CC) results from multiple lymphatic vessel anomalies or thoracic cavity defects and may accompany other congenital anomalies. Fetal chylothorax may increase the risk of death and complications from pleural space lymphatic fluid accumulation, which compromises lung development, pulmonary, and cardiovascular function and from complications arising from the loss of drained lymphatic contents. Prenatal interventions might improve survival in severe cases of fetal chylothorax. The neonatal treatment strategy is generally supportive with interventions that include thoracostomy drainage and attempts to decrease chyle flow using a stepwise approach that begins with the least invasive means. Evidence-based treatment choices are lacking and are much needed. Most cases of CC resolve with time even without specific lymphatic system studies to identify the exact pathology. Expertise in performing lymphatic studies is not universally available. Data on both efficacy and safety of the various therapeutic options are needed to determine the best approach to the treatment of CC.