Background: Intrapericardial extralobar pulmonary sequestration (ELPS) is an exceptionally rare congenital malformation. The location may mimic neoplastic lesions and poses diagnostic and surgical challenges. We present a new case and a systematic review of the literature. Case Presentation: A 3-month-old male infant was referred for evaluation of a congenital intrathoracic mass suspected to be an extralobar sequestration. However, intrapericardial location was not recognized. MRI and CT demonstrated a circumscribed lesion with arterial supply from the right pulmonary artery. Thoracoscopic exploration was attempted but converted to sternotomy. The mass was excised en bloc. Histopathological analysis confirmed extralobar pulmonary sequestration with cystic components, consistent with a hybrid lesion. Postoperative recovery was uneventful. Methods: A systematic literature review was conducted according to PRISMA guidelines across PubMed, Scopus and Embase databases, including only histologically confirmed intrapericardial ELPS. Results: Ten cases were identified. Including the present case, eleven cases have been reported. Prenatal detection occurred in 54% of cases. Fetal demise occurred in two cases due to cardiac tamponade. Aberrant arterial supply originated from the pulmonary arteries in 54% of patients and venous drainage into the right atrium or superior vena cava in 45%. Surgery via sternotomy was performed in all cases with excellent outcomes. Conclusions: Intrapericardial ELPS is an exceptionally rare but surgically curable entity. Early recognition and complete resection are essential to prevent life-threatening complications. This systematic review highlights a consistent vascular pattern supporting its classification as a unique embryologic variant within the CPAM-sequestration spectrum.
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