From a surgical viewpoint, congenital pulmonary adenomatous malformation (CPAM) and bronchopulmonary sequestration (BPS) are the most relevant inborn pulmonary malformations. However, in rare cases, bronchogenic cysts and congenital lobar emphysema also need surgical intervention. CPAM (Fig. 22.1) is considered to represent a hamartomatous change in the tertiary bronchioles, with an incidence of 1 in 25,000–35,000 live births. BPS has an estimated incidence of 0.15–1.7 % in the general population and is defined as a region of lung parenchyma that lacks a normal connection to the tracheobronchial tree and possesses an anomalous systemic blood supply (Fig. 22.2). There is a high proportion of mixed-type lesions, suggesting that CPAM and BPS may have the same developmental ancestry.