We report the case of a 13 year-old girl transferred to our department with a one month history of vomiting, weight loss, and abdominal mass that was diagnosed as Focal Nodular Hyperplasia in an accessory lobe of the liver. Accessory liver lobe is a rare congenital anomaly whose preoperative diagnosis is difficult and usually presents as an incidental finding intraoperatively. Depending on their location and size, they can present as acute abdomen or abdominopelvic mass. In the literature reviewed, we found no association of focal nodular hyperplasia and accessory liver lobe in children.
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