Congenital pulmonary airway malformation (CPAM) is a structural anomaly that occurs during development of the lower respiratory tract. We describe a 4-day-old male infant with this uncommon congenital anomaly. He presented with respiratory distress and low oxygen saturation. A chest radiograph showed infiltration in the right lower lobe, and a chest computed tomography (CT) revealed alveolar opacity with an air bronchogram pattern in the right lung along with mediastinal shift. The right lower lobe was surgically resected. Pathological examination showed an 8-cm, predominantly solid cut surface with a rare tiny cyst, consistent with a congenital cystic adenomatoid malformation (type 3). Congenital pulmonary airway malformations are the most common congenital parenchymal lung anomalies. Although their development is debated, it is believed to result from a halt in fetal bronchial tree growth between the sixth and seventh weeks of fetal life. Flaws in thyroid transcription factor 1 have also been proposed. With the widespread use of high-quality ultrasonography in modern obstetrics, it is now less likely for congenital pulmonary airway anomalies to remain undetected until adulthood. Early surgical excision is generally recommended. However, in asymptomatic infants, management remains controversial because either operative or non-operative approaches may be used later in life, particularly in light of complications such as the potential for mucinous adenocarcinoma with a lepidic-predominant pattern. Patients with this condition in neonatal intensive care units should be managed by a multidisciplinary team that includes pediatric surgeons, neonatologists, and radiologists.