Reports of hepatoblastoma (HB) in preterm infants are quite rare. Herein, we report the clinical management of a preterm infant with inoperable congenital HB. A female fetus that had been diagnosed with a large liver tumor consistent with hemangioma was delivered by emergency cesarean section at 33 weeks of gestation because of fetal distress. Effective antitumor therapy could not be performed, resulting in rapid deterioration and death. The postmortem histopathologic analysis confirmed the tumor as a HB. This report demonstrates the difficulties inherent in both the image diagnosis of HB and in providing efficacious treatments for preterm infants with HB.