SUMMARYCongenital cystic adenomatoid malformation of the lung is a rare lesion that typically manifests as severe progressive respiratory distress in the neonate secondary to expansion of the affected lung. We present a neonate in whom this condition was diagnosed and managed at the Aga Khan University Hospital. In presenting this patient, we hope to highlight the radiological aspects of this condition which, with prompt recognition and management and in the absence of pulmonary hypoplasia, foetal hydrops or other congenital anomalies, has a favourable prognosis.