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This study is to review and retrieve data on adult hepatoblastoma (HB) from English literatures in order to gain a better understanding of this disease. We performed Medline, PubMed (from January 1966 to February 2008), and library searches (National Science and Technology Library, Beijing, China, and Wenzhou Medical College Library, from January 1980 to February 2008) using the key words hepatoblastoma in adult, hepatic tumor, hepatoblastoma and adult. Previously reported HB cases were collected and published reviews were also examined. Fifteen cases that met the search criteria were selected. Review of the cases revealed a slight female preponderance. The patients' age ranged from 17 to 82, with median age of 70 for male and 27 for female. The survival time ranged from two weeks to 38 months, and the median survival time was 6 months. In the articles reviewed, HB presented with non-specific initial symptoms, and the diagnosis was not identified until the tumor biopsy after operation or autopsy. Completely surgical resection is still the major treatment for patients with HB and is considered as the only chance of a better prognosis. Due to the rareness of HB in adults, the choice of treatment should be radical resection if possible, and combined with chemotherapy as adopted in children. HB in the adult is extremely rare and the pre-operative diagnosis is often overlooked. The prognosis is so poor that the awareness of the condition in the differential diagnosis in liver tumors could be beneficial.Key wordsHepatoblastoma; Adult; Diagnosis; Therapy