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Inflammatory myofibroblastic tumor (IMT) of the liver is a rare disease, often confused with hepatic neoplasm. A 60-year-old man underwent liver resection for suspected hepatocellular carcinoma with concurrent appendicectomy for incidentally detected diseased appendix. Histopathology revealed IMT involving both the liver and appendix. We present the diagnostic challenge and approach in a patient with IMT of the liver with the synchronous incidental involvement of appendix treated successfully by liver resection and appendicectomy.