A 64-year-old non-smoking woman with a left upper lobe mass underwent a wedge resection by video-thoracoscopic approach. Intraoperative frozen section examination was unable to rule out a neoplastic disease and a lobectomy was performed. Microscopic analysis showed a typical pulmonary sequestration pattern including arteries with elastic layers in a systemic fashion. Pulmonary sequestration is a development lung disease: upper lobe location is considered uncommon and much controversy exists concerning this form which may be underrated.