Pulmonary sequestration is an unusual congenital malformation characterized by the presence of nonfunctioning lung tissue which usually has no communication with the normal bronchial tree and receives its blood supply from an anomalous systemic artery. Extralobar form is a very rare congenital malformation. We have experienced a 54 year old female patient with a mass in the upper lobe complaining of cough and blood tinged sputum. A triangular shaped mass was located in the left upper lobe, medially. The arterial blood supply were from the thoracic aorta and the pulmonary artery but there was no the tracheobronchial communication. The venous drainage was through the pulmonary vein. The mass was confirmed as extralobar pulmonary sequestration associated with a pericardial defect.