Congenital lung abnormalities are uncommon and diverse in their presentations. However, all those who care for infants and children must have an appreciation for the diagnosis and treatment of these anomalies because the potential consequences can be life threatening. In order to understand the pathophysiology of these malformations, one must have a basic understanding of the embryology of lung development as well as lung anatomy and respiratory physiology, which are presented below. Classical lesions including congenital lobar emphysema (CLE), congenital pulmonary airway malformation (CPAM), pulmonary sequestration, and bronchogenic cysts will be discussed along with several more uncommon lung anomalies.