Pulmonary sequestration (PS) is a rare congenital anomaly that accounts for 1% to 6% of all pulmonary malformations at birth. It is characterized by a focal area of pulmonary tissue that does not have direct communication with the tracheobronchial tree and does not get blood supply from the pulmonary circulation. We present the case of a 28-year-old female with a history of recurrent pulmonary infections who was found to have intralobar sequestration and underwent curative surgical excision. Because pulmonary sequestration is commonly misdiagnosed, as it can mimic other conditions on chest X-rays, this case illustrates the importance of recognizing pulmonary sequestration as a separate entity and diagnosing/treating it appropriately. The patient presented to the hospital with a one-week history of upper chest pain. Chest radiograph showed mild hyperinflated right lung. Computed tomography angiogram (CTA) revealed an 8.9 x 8.3 cm area of hyper-lucency and decreased normal lung architecture in the right lower lobe with an aberrant arterial blood supply suggestive of intralobar pulmonary sequestration. The patient was referred to cardiothoracic surgery and underwent preoperative outpatient pulmonary function testing, which was unremarkable. The patient subsequently underwent successful robotic resection of the right lower lobe sequestration and the pathology report confirmed PS. A diagnosis of pulmonary sequestration is commonly missed, as it can mimic other conditions on chest X-rays. It can present as a solitary nodule or mass, cystic lesion, consolidation, or an air-fluid level. The period between symptom onset and diagnosis is typically more than five years. While digital subtraction angiography is considered the gold standard for imaging, CTA is now preferred because it allows for clear visualization of lung parenchyma and vascular assisting in surgical planning. For our patient, CTA demonstrated a prominent tubular vessel, which showed less enhancement than the opacified pulmonary artery and pulmonary veins, suggestive of an abnormal vascular supply for the right lower lobe sequestration. Management of intralobar sequestration is curative surgical excision. Both video-assisted thoracoscopic surgery (VATS) and posterolateral thoracotomy are viable options for resection. It should be noted that in symptomatic patients, it is recommended to proceed with surgical resection. However, in asymptomatic individuals with intralobar sequestration (ILS), surgical resection is not required but could be considered as prophylaxis to prevent recurrent infections. Asymptomatic individuals with extralobar sequestration (ELS), on the other hand, should undergo serial monitoring as non-operative management is appropriate. This case highlights the importance of including pulmonary sequestration, especially intralobar sequestration in the differential diagnosis of recurrent localized pulmonary infections, especially in a patient who is otherwise healthy. Although rare, it is important to consider this congenital anomaly when evaluating patients with recurrent localized pulmonary infections, chest pain, or hemoptysis., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2024, Pham et al.)