Congenital tracheal anomalies occur in 1:10,000 births and can be associated with congenital cardiac disease. This patient presented with right mainstem atresia, right bronchoesophageal fistula without esophageal atresia (H-type esophageal lung), and left pulmonary arterial (PA) sling. Taking this into consideration, surgical management included right pneumonectomy and placement of expandable prosthesis into the thoracic cavity to buttress the mediastinum and prevent tracheal deviation-induced kinking of the PA sling, which provided pulmonary blood flow to the remaining functional lung. To our knowledge, this is the first reported case of esophageal lung in conjunction with a PA sling, as well as the first documented use of an expandable prosthesis to prevent acute postpneumonectomy syndrome. This case can provide clinicians with increased clinical knowledge for treatment of this rare and potentially lethal combination of congenital anomalies.