Glomerulocystic disease (GCD) is a very rare condition. Only two previous reports have linked this condition with hepatoblastoma. We report a neonate with US evidence of grossly enlarged echogenic kidneys and features typical of hepatic fibrosis, complicated by the presence of a hepatoblastoma. The report discusses the differential diagnosis and highlights GCD as one cause of large, bright kidneys on US. It also adds further evidence to the suggested association between GCD and hepatoblastoma.