Lung malformations are often associated with congenital anomalies in other body districts. The following study analyses the incidence of extrapulmonary involvement in a group of patients born with lung malformations and underlines the importance of investigating the main organ systems. To date, in fact, there is no shared screening system, especially in the neonatal period, on the management of these malformations; it is therefore desirable to create soon a shared protocol.