Autoimmune pancreatitis (AIP) is a distinct subtype of pancreatitis, rare in the pediatric population. Its pathophysiology is poorly understood. A pancreatic focal mass is frequently the clinical presentation, which imposes the differential diagnosis with a pancreatic tumor. This distinction is essential because the treatment of pancreatic tumors is surgical in contrast to the treatment of AIP, which is pharmacological. We describe a case of a 16-year-old girl with AIP who presented with obstructive jaundice, weight loss, fatigue, and a pancreatic mass. This case emphasizes the importance of considering this diagnosis to correctly treat and prevent an abusive pancreatic resection.
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