Congenital defects of the pericardium are rare. Surgical treatment is recommended to relieve symptoms and to prevent cardiac herniation and strangulation. The case of a symptomatic pericardial defect due to a dynamic obstruction of the coronary arteries is described. The diagnosis was suggested by plain chest film, echocardiography, and cardiac catheterization. Confirmation and successful surgical management were accomplished by video-assisted thoracoscopic pericardiectomy.