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Congenital pericardial defects are rare but can cause fatal complications. Most cases are asymptomatic and incidentally detected during a thoracic surgery or autopsy. We report a case of a partial pericardial defect confirmed based on spontaneous pneumothorax. A 16-year-old boy with left spontaneous pneumothorax showed pneumopericardium on chest X-ray. Chest computed tomography revealed a partial pericardial defect. Video-assisted thoracoscopic surgery was performed, and a small pericardial defect was confirmed at the level of the upper p