Congenital cystic adenomatoid malformation (CCAM) is usually observed in neonates because of respiratory distress and may occasionally be observed in older children or adults with recurrent infection 1. This malformation consists of adenomatoid proliferation of bronchioles that form cysts instead of normal alveoli. We report a case of a 11 days old neonate who presented with complaints of severe and persistent respiratory distress. Complete blood investigations, sequential chest radiographs and HRCT thorax of the neonate were performed. This study highlights the role of HRCT in reaching the diagnosis and further guiding the treatment.