Neonatal lymphatic disorders represent a rare group of diseases that arise from the abnormal development of the lymphatic vascular system. Anatomic anomalies or genetic syndromes such as RASopathies are the predominant causes of neonatal lymphatic disorders. Acquired cases, which are less common, can occur secondary to surgical complication or from increased intrathoracic or venous pressure. Infants with severe bronchopulmonary dysplasia (BPD) are at risk of developing lymphatic dysfunction due to chronic ventilator dependance resulting in hyperinflation and increased intrathoracic pressure. While this phenomenon is rare, these cases are likely to increase as survival outcomes in extremely preterm infants continue to improve. Therefore, a deeper understanding of these disorders is needed as lymphatic dysfunction results in significant morbidity and mortality in the neonatal intensive care unit (NICU). This review aims to provide an overview of the classification, clinical manifestations, diagnostic evaluation, and medical management of neonatal lymphatic disease from the perspective of a neonatologist.