We present the case of a Middle Eastern child, diagnosed and treated at 8 months of age for a hepatic neuroblastoma. After surgical removal of a 7 cm mass of the left liver lobe at our institution when the child was 15 months of age, the tumor was reclassified as a teratoid hepatoblastoma. The tumor was composed of fetal and embryonal hepatic tissue, undifferentiated tissue, and a teratoid background of loose mesenchymal tissue containing osteoid, squamous, and mucinous epithelium. We speculate on the histogenesis of teratoid hepatoblastoma and discuss its association with chemotherapy.