An 11-year-old girl presented with abnormal weight gain and was found to have hepatomegaly. MRI of the abdomen revealed a 20-cm hepatic mass. F-FDG PET/CT showed a large hypermetabolic calcified hepatic mass and couple of mildly hypermetabolic pulmonary nodules with associated intrathoracic lymphadenopathy. Liver biopsy was consistent with nested stromal epithelial tumor of the liver, a rare nonhepatocytic, nonbiliary primary neoplasm of the liver associated with variable calcification and ossification.