Abstract
Congenital Lobar Emphysema (CLE) is a rare and life-threatening congenital lung anomaly that often poses a diagnostic dilemma. The problem can be further augmented with co-existent Congenital Heart Defect (CHD) with the left to right shunts. The clinical presentation of congenital heart defects in infantile life is quite similar to the CLE, and CHD being more common gets detected early. The presence of underlying CLE may only be suspected after the repair of the CHD. In our case, a 2-month-old patient presented with respiratory distress, and on evaluation large Ventricular Septal Defect (VSD) and Patent Ductus Arteriosus (PDA) were detected. After the successful repair of the cardiac defects, the infant continued to have cough, respiratory distress, and failure to thrive. Then on radiological re-analysis CLE was suspected on X-ray, and confirmed by Computed Tomography (CT). CLE can get detected on an X-ray chest, but it is often confusing and misleading at an early stage.