Abstract
Pancreatic lithiasis, the formation of calcifications in the pancreatic duct, occurs uncommonly in pediatric patients but can occur more frequently with chronic pancreatitis (CP). Cystic fibrosis (CF) is one of the major causes of pancreatic lithiasis in pediatric patients, with mutations in the CF transmembrane conductance regulator (CFTR) gene reported in up to 23% of pediatric CP patients. Mutations in the CFTR gene can lead to mild cases of CF, which may delay diagnosis and treatment. In such cases, pancreatitis can be the presenting symptom in children with CF. We report a unique case of a 10-year-old female with previously undiagnosed and untreated CF presenting with abdominal pain, vomiting, and obstructive jaundice. Her pancreatic lithiasis and biliary obstruction were successfully treated with endoscopic retrograde cholangiopancreatography (ERCP).