Variant scimitar syndrome with intralobar pulmonary sequestration containing adenocarcinoma

Scimitar syndrome is a rare congenital anomaly in which the right lung is hypoplastic and a scimitar vein is draining into the inferior vena cava. It is known to be sometimes complicated with pulmonary sequestration. However, a right single pulmonary vein draining normally into the left atrium is rare. Here, we report a case of variant scimitar syndrome complicated by intralobar pulmonary sequestration containing an adenocarcinoma. In this case, the aberrant arteries from the abdominal aorta were ligated and the pulmonary sequestration containing adenocarcinoma was completely removed through wedge resection. Twenty months after the surgery, the patient was alive without cancer recurrence. An elaborate preoperative plan is important for safety in cases with vascular malformation.