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Background: Asymptomatic congenital pulmonary airway malformations (CPAMs) and congenital lobar emphysema (CLE) may safely be observed, though little is known about how many patients later require surgery. The management of these lesions remains controversial, as limited research exists on which patients later require surgery. Notably, there are few reported cases where patients become symptomatic and require definitive treatment beyond childhood., Case Description: We present the rare case of a 17-year-old female who developed dyspnea on exertion associated with pre-syncope, following diagnosis of a CPAM at birth. She had undergone surveillance until 2 years of age. The decision to proceed with a left upper lobectomy via thoracotomy was made, for treatment of her symptoms. During the surgery, one lung ventilation suggested that oxygenation had likely limited to the patient's healthy lung, prior to intervention. The surgery was uncomplicated, and her recovery was uneventful. Pathology revealed CLE., Conclusions: This case highlights that congenital lung malformations should remain in the differential diagnosis of patients presenting in adolescence or adulthood with new onset respiratory symptoms. For patients with a history of untreated congenital lung pathology, it is important to consider the presence of adhesions and loss of domain in perioperative planning. This case also highlights the ongoing need for research on the prognostication of these lesions to better inform surveillance., Competing Interests: Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://acr.amegroups.com/article/view/10.21037/acr-24-28/coif). D.G.F. reports that he has been the facilitator of a physician advisory meeting for AstraZeneca. The other authors have no conflicts of interest to declare., (2024 AME Publishing Company. All rights reserved.)