Abstract
Congenital lobar emphysema (CLE) is a rare and life-threatening lung anomaly that often poses a diagnostic dilemma. Co-existent congenital heart defects (CHD) with left-to-right shunts can further complicate the diagnosis of CLE. The presence of underlying CLE may only be suspected after the repair of CHD. Here, we discuss a 2-month-old patient who presented with respiratory distress and was identified to have a large ventricular septal defect (VSD) and patent ductus arteriosus (PDA). After the successful repair of the cardiac defects, the infant continued to experience respiratory distress and failure to thrive. CLE was subsequently diagnosed on imaging.