Patients with Beckwith-Wiedemann Syndrome (BWS) are predisposed to developing hepatoblastoma. Clinical data were reviewed in all cases of hepatoblastoma in patients with BWS reported in the literature and from personal cases. Patients were identified by literature review using PubMed and by a search of the authors’ local tumor registries. Fifty-six patients were identified. The median age of presentation with hepatoblastoma was 6 months (range birth-30 mo). Thirteen of 26 patients were born prematurely. Of 31 evaluable patients, 19 exhibited hemihypertrophy. Thirty-two of 33 patients with α-fetoprotein data reported had elevated levels at diagnosis. Overall survival was 75% (27 of 36 patients). Of 25 patients with data who survived, 24 were treated with chemotherapy and surgery (vs. only 2 of 8 who did not survive). All 9 patients with hepatoblastoma detected by routine screening with outcomes reported were surviving at the time of the reports. Overall survival was high in patients with BWS and hepatoblastoma, especially given lower stage at presentation and when treated with surgery and chemotherapy. Future prospective trials should evaluate if BWS is independently associated with outcome and if the outcome is improved by routine screening.