Congenital cystic adenomatoid malformation of the lung is a rare but potentially lethal variety of congenital pulmonary defect. This is a multicystic mass of dysplastic pulmonary tissue in which there is proliferation of bronchial structure at the expense of alveoli. We report a newborn infant with respiratory distress. The chest X-ray showed multicystic lesions with air-fluid level and shift of mediastinum. The patient was successfully treated with segmentectomy. The clinical manifestations, radiographic and pathologic features were summarized and the literature reviewed.