Pulmonary sequestration is an abnormal lung tissue that does not communicate with tracheobronchial tree, and that receives blood supply from anomalous arteries. Pulmonary sequestration is divided into two anatomical forms, intralobar and extralobar. Extralobar pulmonary sequestration is usually located between the diaphragm and the lower lobe and has systemic arterial supply and venous drainage, and most patients are diagnosed in their infancy. We report an extralobar pulmonary sequestration located between the right upper lobe and the lower lobe in a 48-year-old female adult, which has anomalous blood supply from the right pulmonary artery and venous drainage directly into the left atrium.