Surgery is usually recommended for pulmonary segregation, but may be considered without surgery if respiratory function is not affected. Pulmonary segregation is a congenital malformation of lung development in which a portion of the lung tissue is separated from the normal lung and develops separately and receives blood supply from the circulation, resulting in a cystic mass in the lungs that has no respiratory function. It can be divided into intrapulmonary and extrapulmonary isolation. Intrapulmonary segregation is common, usually non-specific, and mostly symptomatic when combined with respiratory infections. Intrapulmonary isolation can have recurrent secondary infections and surgical treatment is usually recommended; extrapulmonary isolation, if it does not traffic with the gastrointestinal tract and is asymptomatic, can be left untreated. Therefore, the surgical treatment of pulmonary isolation needs to be based on the patient’s condition. If pulmonary isolation is diagnosed, early and standardized treatment is recommended to reduce the adverse effects of the disease.