Cystic fibrosis (CF) is the most common severe autosomal recessive disorder in Caucasians. A homozygous change of the CF transmembrane regulator gene causes viscosity in pancreatic and bronchial secretion. Obstruction of secretion is responsible for liver and biliary tract symptoms. Congenital cystic adenomatoid malformation (CCAM) is a rare lung disorder of unknown etiology affecting the distal bronchi. It is important because of the risk of recurrent lung infections and malignancy. A 3-month-old female patient was brought to our hospital with symptoms of insufficient weight gain and pale appearance. The patient was hospitalized with a pre-diagnoses of malnutrition, hemolytic anemia, and cholestasis. She was diagnosed with CF liver disease and congenital adenomatoid malformation following thorough evaluation. Our patient with CF liver disease and pancreatic involvement was found to have a deltaF508 homozygous change. Our case is reported because of the rare association of hereditary CF disease and CCAM of unknown cause.