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Congenital pulmonary airway malformations (CPAMs) are rare multicystic lung lesions typically diagnosed antenatally. We present a case of a term female neonate with antenatally diagnosed CPAM who required pleuro-amniotic shunting at 22 weeks of gestation. The patient was born with a right-sided pneumothorax and severe cardiorespiratory distress, necessitating extracorporeal membrane oxygenation (ECMO). Chest CT confirmed CPAM, revealing multiple cystic lesions in the right middle lobe and a significant contralateral mediastinal shift. On the second day of life, while on ECMO, the patient underwent a right middle lobectomy and an upper lobe anterior segmentectomy via a posterolateral thoracotomy. Post-surgery cardiac CT showed narrowing of the left pulmonary artery, although a perfusion study indicated normal left lung perfusion. Histopathological examination identified CPAM type 1 with invasive mucinous adenocarcinoma (IMA; stage 1: pT1b), featuring low-to-intermediate cellularity and KRAS G12D mutations. The invasive mucinous component measured at least 15 mm but did not invade the visceral pleura. After a gradual weaning process, the patient was successfully extubated and discharged home after 70 days. To our knowledge, this is the first reported case of CPAM type 1 with IMA that underwent pleuro-amniotic shunting in the second trimester., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Gopalaswamy et al.)