This chapter discusses the spectrum of congenital lung disease from the upper airway down to the lung parenchyma and microvasculature, and associated relevant malformations in the chest wall and mediastinum, and systemically. A systematic way of describing an individual malformation is proposed, using clear words to delineate the components of the malformation before planning treatment. Congenital lung disease may present in utero right up to old age. Many large malformations diagnosed antenatally largely regress in the third trimester of pregnancy, and are only detectable postnatally on computed tomography (CT) scanning. Management of asymptomatic congenital cystic malformations is controversial; many remain symptom free for a long time, whereas others become the seat of infection or malignancy, or result in other complications, such as air embolism. Histological overlap between what were once thought of as discrete entities, such as congenital cystic adenomatoid malformation and sequestration, are common, and attempting to determine histology from clinical images is fraught with difficulty. Newer imaging techniques, such as magnetic resonance and CT angiography, are increasingly used to image congenital lung malformations, with conventional angiography reserved for situations when therapeutic embolization of the malformation is being considered. Advances in surgical techniques, in particular for congenital diaphragmatic hernia, mean that there are more survivors into childhood and adult life. The optimal follow-up for patients with congenital lung malformations, whether surgically treated or not, remains to be determined.