Introduction: Congenital cystic lesions of the lung are uncommon but share similar embryologic and clinical characteristics. An overall incidence of congenital cystic lesions of the lung is 1/10,000 to 1/25,000 births (and 2.2% as compared to acquired lesions). Presentation varies from life-threatening symptoms at birth to incidental findings at autopsy. Diagnosis is either made in utero or due to complications of the lesion, such as lung abscess, pneumonia, or pneumothorax