Teratoid hepatoblastoma is a rare histologic subtype accounting for 4% to 10% of all hepatoblastomas and is characterized by the presence of divergent differentiation, including squamous, mucinous, melanocytic, cartilaginous, osseous, skeletal muscle, and neural elements. It is postulated that the teratoid elements might derive from multipotential less-differentiated stem cells. Teratoid hepatoblastoma responds poorly to chemotherapy and therefore recognition of this variant has prognostic implications. We describe a 1½-year-old child with teratoid hepatoblastoma characterized by unusual clinical and morphologic patterns including prominent neuroendocrine and squamous differentiation.