Background: Liver is prone to different diseases including metabolic, congenital, infections, drug injuries, and malignancies. Liver biopsy is an aggressive but definitive way to diagnose liver diseases.Objectives: The present study is designed to evaluate the results of liver biopsies in children referred to the Pediatric Gastroenterology Department of Nemazee Hospital.Materials and Methods: The present retrospective study was conducted on 308 liver biopsy specimens from children suspected to liver diseases who had been referred to Pediatric Gastroenterology Department affiliated to Shiraz University of Medical Sciences between March 2003 and March 2008.Results: Totally 308 liver biopsies from the children aged less than 18 years was investigated. There were 128 girls and 180 boys with mean age of 6.04±5.97 years and Male/Female ratio of 1.4/1. The most common pathologic findings were chronic hepatitis (n=71; 23.1%), metabolic diseases (n=37; 12.1%), cirrhosis (n=27; 8.8%), secondary hemochromatosis (n=22; 7.1%), neonatal hepatitis (n=22; 7.1%), and hepatic malignancies (n=21; 6.8%). Liver biopsy was non diagnostic in 16.2% (n=50) of the patients.Conclusions: Chronic hepatitis, metabolic liver diseases, cirrhosis, and neonatal hepatitis are the most common liver diseases in children in our center. In this study a high percentage of liver biopsies were non-diagnostic. Therefore, providing the necessary facilities and equipment for special assessment of liver tissue are essential in this center.