Congenital malformations of the lung are rare and vary widely in their presentation and severity. The most common manifestation of the congenital cystic disease of the lung at newborn and early infancy is respiratory distress. Later on in life, cysts usually lose this compressive character and may remain asymptomatic until infection occurs, while producing cough, dyspnea and thoracic pain. The purpose of this study is to review authors institutional experience of congenital cystic lung disease, with specific reference to diagnosis, treatment, as well as outcome, furthermore, to present some cases with unusual clinical manifestations.