Pulmonary sequestrations are rare congenital anomalies with an incidence of 0.15% to 1.7%, and they are divided into intralobar and extralobar pulmonary sequestrations (ELPS). 1 A nonfunctioning pulmonary tissue characterizes them without normal connection to the tracheobronchial tree and supplying blood from 1 or more abnormal systemic arteries. Most ELPS are located between the lower lobe of the lung and the diaphragm; however, they may occur anywhere within the thorax and partially or completely within the diaphragm. 1,2 In approximately 25% of patients with ELPS, different congenital lung abnormalities, such as hypoplasia, congenital cystic adenomatoid malformation, congenital lobar emphysema, or bronchogenic cyst, associated with ELPS are known. 2 In this report, we present a child with an ELPS located in the upper posterior mediastinum associated with the azygos lobe.