Academy of Medical Sciences of I.R. Iran Archives of Iranian Medicine 1029-2977 23 9 2020 09 01 A Case of Congenital Hepatoblastoma Coexisting with Pulmonary Hypertension 621 623 10.34172/aim.2020.73 EN Zeynep Canan Özdemir Adviye Çakıl Sağlık Yeter Düzenli Kar Pelin Köşger Deniz Arık Ayşe Neslihan Tekin Özcan Bör Journal Article 10.34172/aim.2020.73 2018 11 19 2019 03 16 Hepatoblastoma (HBL) is the most frequently detected malignant tumor of the liver in childhood. HBLs detected antenatally or up to 3 months after birth are considered congenital HBLs. We report a five-day-old female infant in whom a hepatic mass was detected at 20 weeks’ gestation. At birth (36 weeks), the hepatic mass measured 12x6 cm, and she had respiratory distress. Pulmonary hypertension (PHT) was detected on echocardiographic evaluation. Despite dual medical therapy, her PHT did not improve. Histologically, the biopsy demonstrated a mixed epithelial-mesenchymal HBL with predominance of fetal morphology in the epithelial component. Chemotherapy was initiated on postnatal day 15; however, the baby died of respiratory failure on postnatal day 23. Conclusion: HBL is an embryonal tumor which can develop early in the intrauterine period. Although the mechanism is not known, it may cause PHT which would affect the prognosis negatively.