Background: Cystic fibrosis (CF) is the leading etiology for exocrine pancreatic insufficiency (EPI) in children, followed by chronic pancreatitis, and other genetic disorders. Pancreatitis has historically been considered rare in CF due to pancreatic parenchymal loss early in infant development. However, newborn screening, advancements in genetics, and CF Transmembrane Conductance Regulator (CFTR) modulator therapies have all increased the proportion of CF patients with pancreatitis. Newborn screening helps to identify CF patients even before symptoms may manifest. The cystic fibrosis transmembrane conductance regulator gene, which encodes a chloride and bicarbonate channel expressed in the apical membrane of epithelial cells. The gene is expressed in multiple organ systems, which explains the wide variety of medical conditions experienced by people with CF, affecting the pulmonary, endocrine, gastrointestinal, pancreatic, biliary and reproductive systems. Conclusion: Pancreatic insufficiency common in children with cystic fibrosis so early diagnosis and important management improve outcome of patients regarding gaining weight and decrease pulmonary exacerbation as result decrease mortality. recommend to cases with positive in prenatal screaning and pancreatic sufficient to follow up.