Pulmonary agenesis is a rare congenital anomaly that can present in the neonatal period or later in life. It frequently causes respiratory distress in the neonatal period. Nearly half of the cases are associated with cardiovascular, gastrointestinal and musculoskeletal malformations. Total atelectasis, congenital diaphragmatic hernia, congenital cystic adenomatoid malformation, pulmonary sequestration, chylothorax, bronchogenic cyst, and chest tumors are included in the differential diagnosis. Computed tomography scan of the chest is the investigation of choice to define the pulmonary anatomy. Prognosis depends mainly on associated anomalies and pulmonary function. Herein, we report an infant with unilateral pulmonary agenesis in the neonatal period, and we discuss the associated anomalies and course of this rare anomaly.

Keywords: yenidoğan, pulmoner agenezi.