Congenital cystic diseases of the lung are rare but their incidence is increasing, especially with the widespread use of antenatal ultrasound. Close relationships between airway and respiratory segments and vascular structures during embryonic development can lead to malformations. It should be remembered that congenital cystic lung diseases have the potential to be life-threatening and the possibility of missed diagnosis is high. Surgical resection yields good results in symptomatic lesions, with lobectomy being the most commonly performed surgical approach. Some centers recommend surgical resection even in asymptomatic patients to prevent infection and exclude malignancy.