A major hallmark of neurofibromatosis type I (NFI) is the development of benign tumors. Hepatoblastomas associated with NFI is reported rarely in the literature. We present here a one year-old girl with both NFI and hepatoblastoma. Hepatoblastoma can be associated with NFI. Abdominal ultrasound is not included within the diagnostic criteria or surveillance of NFI. As NFI can be associated with hepatoblastoma and other abdominal tumors, a careful physical examination and an abdominal USG if necessary should be considered.