Congenital cystic adenomatoid malformation is one of rare pulmonary hypoplastic diseases. It has been subdivided into 3 types (I-III). Respiratory distress and hydrops are usually diagnosed pre- or postnatally by ultrasonography or radiography. The pathogenesis of CCAM has not been clarified yet. Here, we present an unusual case of bilateral CCAM (type III) of a 2-month-old infant who died suddenly. Concomitant lung malformation and fatty degeneration in hepatic cells make this case unique since such malformation is seldom found in type III CCAM.