Abstract
<title>Abstract</title> <p> <bold>Background</bold> Pulmonary agenesis is a rare congenital malformation that occurs due to a defect in lung bud development. It presents with respiratory distress and is often mistaken for common respiratory conditions, including pneumonia, particularly in resource-limited settings. This leads to delayed diagnosis and inappropriate care. Therefore, reporting such cases from resource-limited settings is important to enhance clinical awareness and improve outcomes. <bold>Clinical Case</bold> We present a term female neonate who experienced severe respiratory distress shortly after birth. Initially, the patient was treated for pneumonia at a health center. However, her condition worsened, and she was referred to a tertiary hospital. At admission, the patient was in severe respiratory distress. Physical examination revealed severe intercostal and subcostal retractions as well as absent air entry on the left hemithorax. Chest radiography showed a complete homogeneous opacification of the left hemithorax with ipsilateral rib crowding; and hyperinflation of the contralateral lung. Contrast-enhanced chest tomography confirmed the absence of the left lung, left main bronchus and left pulmonary artery. Transthoracic echocardiography revealed enlargement of the right-sided heart chambers, a moderate patent ductus arteriosus with bidirectional shunting, and severe pulmonary hypertension. The patient was supported with continuous positive airway pressure (CPAP) and medical therapy for pulmonary hypertension and cardiac support. At 6 months of age, her clinical condition gradually improved but remained oxygen-dependent. <bold>Conclusion</bold> Clinicians should consider this rare congenital anomaly in children presenting with persistent respiratory distress, asymmetric chest findings and order appropriate imaging. </p>