This rare embryonal Tumours originates from immature liver cells. It usually presents in children under 3 years of age, is more common in males than in females, and shows no association with hepatitis B infection or cirrhosis. The majority of cases of hepatoblastoma are sporadic, although familial cases occasionally occur (Hartley et al. 1990). Congenital abnormalities, particularly abnormalities of the urogenital system, are more common in children who develop hepatoblastoma. Syndromes associated with an increased susceptibility to hepatoblastoma development include Beckwith– Wiedemann syndrome and congenital hemihypertrophy, caused by dysregulation of the 11p15 growth region. Risk of neoplasia in children with this disorder is increased if hemihypertrophy is present, and children with BWS and loss of methylation at KvDMR1 appear to be at higher risk of hepatoblastoma than Wilms’ Tumours (Cooper et al. 2005). It is not a component of another overgrowth syndrome, Bannayan–Riley–Ruvalcaba syndrome. Hepatoblastoma may occur in children with familial adenomatous polyposis (Herzog et al. 2000). Hepatoblastoma surveillance for individuals at increased risk is controversial. Some clinicians advocate 3 monthly physical examinations, serum α-fetoprotein, and abdominal ultrasound until the age of 3 years and then 6 monthly until 6 years of age (Chitayat et al. 1990a, b; Clericuzio et al. 2003). However, there is little evidence for the efficacy of this screening program, and currently the potential benefits and risks are poorly understood.