This case report attempts an approach to the clinical findings of hepatobiliary manifestations in Cystic Fibrosis. Infant less than 1-month-old with an insidious clinical picture that debut with hepatobiliary manifestations and jaundice, upper respiratory infection and gastrointestinal sepsis non-specific. Cystic Fibrosis is the most frequent autosomal recessive clinical condition in Caucasians. It is associated with liver involvement around 30%. In children, hepatobiliary symptoms occur at puberty when damage to the liver system is in advanced stages. The atypical presentation of Cystic Fibrosis with liver involvement is very rare and lethal. Understanding the different form of Cystic Fibrosis, it is essential for early diagnosis and to achieve integral management.