Hepatobiliary cystadenoma is a rare hepatic neoplasm that has been reported only 10 times in the pediatric population. Although considered a benign cystic tumor of the liver, hepatobiliary cystadenoma has a high risk of recurrence with incomplete excision and a potential risk for malignant degeneration. Complete tumor excision with negative margins is the mainstay in treatment. Unfortunately, due to the paucity of cases and its vague presentation, hepatobiliary cystadenoma is rarely diagnosed preoperatively. Therefore, in patients with hepatic cystic masses without a clear diagnosis, total resection of the lesion with negative margins is indicated to adequately evaluate for malignant potential and limit the risk of recurrence. We describe a 2-year-old girl with an asymptomatic abdominal mass that was found to be hepatobiliary cystadenoma. In addition, the pathogenic, histopathologic and clinical features of hepatobiliary cystadenoma are reviewed
