Congenital cystic adenomatoid malformation of the lung is a rare lesion which manifests as neonatal respiratory distress secondary to progressive expansion of the affected lung. Three distinct types have been described based on the size of the cysts and the microscopic appearance. The treatment of choice is excision of the affected lobe. The prognosis is favourable in the absence of pulmonary hypoplasia, foetal hydrops, or associated congenital anomalies.