AbstractA major hallmark of NF1 is the development of benign tumors, including peripheral neurofibromas, plexiform neurofibromas, gliomas of the optic tract, other low grade gliomas, and pheochromocytomas. Hepatoblastoma have not been previously reported in patients with neurofibromatosis type 1. We present a case of a 9‐month‐old boy diagnosed with both hepatoblastoma and neurofibromatosis type 1. Hepatoblastoma occurs in association with several well‐described cancer predisposition syndromes, including familial adenomatous polyposis, Beckwith–Wiedemann syndrome, Li‐Fraumeni syndrome, trisomy 18, and glycogen storage disease type I. This paper describes a case of hepatoblastoma diagnosed in association with neurofibromatosis type 1. Pediatr Blood Cancer 2007;49:357–359. © 2005 Wiley‐Liss, Inc.