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Background: Inflammatory myofibroblastic tumors (IMTs) are rare with unknown etiology. As pancreas involvement is rare in IMTs, here, we report a case of a girl with IMT, referred to our hospital. Case presentation: A 4-year-old girl presented with chief complaints of generalized itching and jaundice. Abdominopelvic computed tomography (CT) scans with contrast showed a homogeneous isodense mass lesion in the head of the pancreas with a compressive effect on the distal part of the common bile duct (CBD). Dilatation of intrahepatic bile ducts and CBD (8mm) was observed. Magnetic resonance cholangiopancreatography (MRCP) examination showed dilated gallbladder without stones and intrahepatic/extrahepatic bile ducts. The CBD was dilated and a mass was found in the head of the pancreas. Immune-histochemical studies revealed spindle myofibroblastic tissues with lymphoplasmacyte and eosinophil infiltration. All of them were compatible with pancreatic IMTs. The surgery improved the symptoms. Conclusions: The IMTs of the pancreas can have symptoms like pancreatic cancer. The careful evaluation by imaging and pathology is recommended.