Cystic adenomatoid malformation involving an entire lung in a 22-year-old woman

Congenital cystic adenomatoid malformation is an uncommon cause of respiratory distress in infants and is a rare entity in adults. Presentation in older patients is that of recurrent pulmonary infections. Usually a single lobe is involved. This report describes congenital cystic adenomatoid malformation involving the entire right lung in a 22-year-old woman presenting with gastrointestinal bleeding due to cavernous transformation of the portal and splenic veins.