Focal nodular hyperplasia of the liver is a benign tumor that usually affects young women. Traditionally, its treatment in children has been conservative. As a result of its rarity in childhood, its differential diagnosis with other liver tumors is challenging. We present the case of a 5-year-old girl with a 1-week history of fever and abdominal pain. No definite diagnosis could be obtained after serial imaging and liver biopsy. As a result of uncertainty in the imaging and needle biopsy results, the patient underwent complete tumor resection. Pathology showed focal nodular hyperplasia that affected the right lobe of the liver. After surgery, the child was doing well at 24 months of follow-up.
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