Purpose of the Review <p>Chylothorax is the most common cause of neonatal pleural effusions. Although rare, neonatal chylothorax poses a significant risk for morbidity and mortality for these patients. Additionally, heterogeneity in the etiology of chylothorax and the variability in diagnostic and therapeutic techniques used by clinicians make standardization of evidence-based treatment guidelines difficult. The purpose of this review is to explore the existing and most recent literature on neonatal chylothorax to describe the diagnostic, imaging, and therapeutic modalities that are available to clinicians.</p> Recent Findings <p>In the majority of neonatal chylothorax cases, symptoms improve with nutritional and pharmacologic therapy alone. In recent years, there has been a transition in focus to minimally invasive imaging and interventional techniques for neonatal chylothorax resistant to conservative measures. In particular, the role of dynamic magnetic resonance lymphangiography and minimally invasive or microsurgical techniques such as thoracic duct embolization or lymphovenous anastomosis are being explored.</p> Summary <p>The goal of neonatal chylothorax management is to stop chyle accumulation by the most conservative and least invasive method possible. Larger, multicenter studies will be important to develop evidence-based treatment guidelines and improve outcomes in these patients.</p>