Both hepatoblastoma and neuroblastoma are occasionally associated with congenital syndromes such as Beckwith–Wiedemann syndrome and trisomy 18. There have been no reports of hepatoblastoma in patients with Kabuki syndrome, whereas one patient with neuroblastoma and this syndrome has been reported. In this paper we present two patients with Kabuki syndrome and a neoplasm: a child of 6 years with hepatoblastoma and an infant, of 6 months affected by neuroblastoma. © 2010 Wiley-Liss, Inc.