Congenital cystic adenomatoid malformation (CCAM), pulmonary sequestration, congenital lobar emphysema (CLE), and bronchogenic cysts are all congenital malformations of the lung that present in imaging studies as abnormal air, air/fluid, or fluid-filled cysts. The embryology, histology, clinical presentation, and treatment of these lesions are discussed based on world literature and our experience with 22 operative resections of congenital lung cysts over the past 10 years. The roles of prenatal diagnosis and fetal surgery in the management of certain lung cysts are considered. Computed tomography has emerged as an extremely useful tool in the differential diagnosis of these lesions. Surgical excision by segmentectomy or lobectomy for intrapulmonary lesions and simple excision for extralobar sequestrations and bronchogenic cysts are safe and have a low incidence of complications. Asymptomatic patients with CLE may not require resection. Overall, with accurate diagnosis and preoperative planning these rare but fascinating anomalies can be treated safely and effectively, with excellent results.