The first description of what is now known as lung sequestration is attributed to J. J. Huber in 1777. The term sequestration was introduced by D. M. Pryce in 1946. Pulmonary sequestration may be defined as a segment of the lung that has no macroscopically identifiable communication with the tracheobronchial tree. The most widely used concept explains pulmonary sequestration as a formation of an accessory lung bud caudal to the normal lung buds. Normally, the sequestration is perfused by a systemic artery arising from the descending aorta. The intrapulmonary sequestration is integrated in the normal lung with a predominance in the left lower lobe. The extralobar sequestration is isolated from the normal lung, located between the lung and diaphragm, in the mediastinum, or intra-abdominally. The extralobar sequestration drains into systemic veins and is mostly combined with other malformations, such as diaphragmatic hernia. With an incidence of 0.15–1.8 %, pulmonary sequestration is one of the most common congenital lung anomalies. Intrapulmonary sequestration is diagnosed in adults, where chronic pneumonia in the sequestration induces further investigations. The surgeon has to realise the atypical vascular anatomy. Extrapulmonary sequestration with other anomalies is detected early in life. Surgical treatment comprises resection of the sequestration and correction of the combined defects like diaphragmatic hernia.