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Diagnosis and management of gallbladder disease is a core component of the practice of most adult general surgeons. While gallbladder disorders are less frequent in infants, children and adolescents, their pathophysiology, presentation, and natural history is often distinct from that in adults. Various congenital disorders and diseases that disproportionately affect children can impact bile metabolism and excretion and can lead to biliary tract findings and symptoms. Management usually mirrors that in adults, but important distinctions exist and it is important for a pediatric surgeon to recognize these unique aspects of pediatric gallbladder disease.