Abstract. A preterm infant with signs of non-immune hydrops was born at 30 weeks gestation to a 34-year-old mother after an uneventful pregnancy with normal antenatal fetal ultrasound scans. Physical examination revealed generalized hydrops with hepatomegaly and ascites. Extensive investigations identiﬁed an elevated -fetoprotein. An abdominal ultrasound scan showed a huge primary solid mass in the liver that compressed the inferior vena cava, with evidence of signiﬁcant arterio-venous shunting suggestive of hepatoblastoma. Liver biopsy conﬁrmed the diagnosis of hepatoblastoma with a fetal epithelial pattern.