INTRODUCTIONPleural effusion is an abnormal collection of fluid within pleural space usually resulting from excess fluid production and or decreased lymphatic absorption. Pleural effusion is rare during neonatal period with the estimated prevalence of 0.06%1. Neonatal pleural effusion may be due to antenatal congenital causes or postnatal acquired causes. Antenatal pleural effusion may be due to hydrops fetalis, congenital chylothorax, congenital heart disease (CHD), chromosomal anomalies, pulmonary anomalies or infection like congenital herpes simplex viral (HSV) and Paro virus infection. Postnatal acquired causes include extravasation of peripherally inserted central catheters, parapneumonic effusion or traumatic chylothorax.As neonatal pleural effusion can cause significant respiratory distress, vigilant delivery room management is required. Identifying the underlying cause is important as it leads to specific therapeutic measures. Diagnostic chests tap for pleural fluid analysis is important for diagnosis and its consequent management.2Similarly, echocardiography, karyotyping and other investigations are also indicated for identifying subsequent etiology.Here, we report a case of neonatal pleural effusion; which on evaluation; pulmonary sequestration-extra lobar type was found as the cause of effusion. Extra lobar pulmonary sequestration associated with pulmonary effusion is a very rare entity.3 The purpose of this case report is to familiarize clinician regarding lung sequestration as the cause of neonatal pleural effusion and prevent subsequent morbidity and mortality.