Abstract: Objective To explore the clinical features of congenital hepatic fibrosis (CHF) in children. Methods A total of 111 patients diagnosed with CHF during January 2002 to June 2015 were included and divided into children group and adult group according to the age at diagnosis. The clinical data including gender, age, clinical symptoms, signs, laboratory examinations, and imaging characteristics were retrospectively analyzed and compared between two groups. Results There were high incidences of splenomegaly and liver cirrhosis in both children and adult groups, and no difference was found between two groups (P > 0.05). The fatigue, jaundice, and hepatosplenomegaly were more common in children group while the renal cyst and liver cyst were more common in adult group, and the differences were statistically significant (P all < 0.05). Compared with adult group, the abnormal rate of ALT, AST, ALP, LAP, TBA, and AST were significantly higher in children group (P all < 0.05). Conclusion CHF patients often have hepatocirrhosis and splenomegaly. CHF patients diagnosed in childhood have more sever hepatosplenomegaly, are more in high levels of ALT, AST, ALP, LAP, and TBA, and less in renal cyst and hepatic cyst.