AbstractLittle is known about the etiology of hepatoblastoma. Because of the young age at diagnosis, several studies have looked at various birth characteristics. The purpose of our study was to investigate the incidence of hepatoblastoma in the Nordic countries and the association between selected birth characteristics and hepatoblastoma. Data from national cancer registries and birth registries in Denmark, Sweden, Norway and Finland 1985–2006 was used. Overall, 155 children with hepatoblastoma aged 0–14 years were included and individually matched to five controls drawn randomly from national population registries. The incidence rate of hepatoblastoma was 1.7 per million person‐years with a predominance of boys (1.5:1). Incidence rate was highest before the age of 1 year (8.3 per million person‐years). A higher risk of hepatoblastoma was found in children with birth weight <1,500 g [odds ratio (OR) = 9.5; 95% confidence interval (CI): 2.3–38.2], born preterm in week 22–32 (OR = 4.5; CI: 1.8–11.5) and Apgar scores <7 after 1 min (OR = 3.1; CI: 1.3–7.1) and 5 min (OR = 7.5; CI: 1.8–32.4). A doubling in risk was found in children who were large for gestational age (OR = 2.3; CI: 1.0–5.3). No associations were found with birth order, maternal age or maternal smoking. Our study indicates that intrauterine and/or neonatal factors are associated with increased risk of hepatoblastoma. These may include low birth weight and asphyxia leading to neonatal intensive care. Alternatively, the factors may be a consequence of hepatoblastoma developing in utero.