Background: Hepatocellular carcinoma is a rare tumor in children, thus, there are not many studies regarding clinicopathologic findings of this tumor and no report regarding this tumor in this age group from Iran. Objectives: In this study, we tried to find out the clinicopathologic findings including outcome of hepatocellular carcinoma in the patients under the age of 18. Methods: During the last 10 years (2005 -2016), we collected all hepatocellular carcinoma cases in the affiliated hospitals of Shiraz University of Medical Sciences (as the largest referral center in the South of Iran and the only center of pediatric liver transplantation in the whole country) from the archives of Pathology department. Next all clinical data were subsequently extracted from the patients' hospital charts. Results: We collected 30 cases of hepatocellular carcinoma with the age range of 9 months to 18 years with no significant sex preference. All of the patients, except 3, showed abnormal levels of AFP. The most common underlying cause of liver tumorigenesis was type I tyrosinemia. Conclusions: Hepatocellular carcinoma in children is a rare tumor and is mostly secondary to an underlying liver disease, which is tyrosinemia in our center. Hepatitis B related hepatocellular carcinoma is rare in our country, which seems to be related to successful early neonatal vaccination against this viral disease. This tumor has a poor outcome in children and without liver transplantation, has a very short survival rate.