The clinical and pathologic features of 54 children with hepatoblastoma are reviewed. Their average age at diagnosis was 17 months (range: 4 months–4½ years) and there was a predilection for males in a ratio of nearly 2:1. Unusual manifestations included isosexual precocity (two cases), hemihypertrophy and Budd-Chiari syndrome (one case), and synchronous Wilms' tumor (one case). Thirty-two of 49 conventional hepatoblastomas were subclassified as epithelial type (19 predominantly embryonal, 13 predominantly fetal), and 12 had mixed epithelial–mesenchymal histology. Pathologic material in five cases was too limited for reliable subclassification. Hepatoblastomas classified as anaplastic type had more primitive histology with a predominance of small- to medium-sized cells in diffuse sheets.