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Abstract (summary): Congenital Hepatic Fibrosis (CHF) is histologically characterized by increased profile of dilated biliary tracts and progressive periportal fibrosis. We describe for the first time the change in microRNA expression pattern in human CHF liver tissues using miRNA-sequencing analysis. The expression pattern of miRNAs in the CHF patient liver is distinguishable from that of normal and other typical forms of hepatic fibrosis by differential analysis. We have isolated, for the first time, diseased biliary epithelial cells from patients with human Congenital Hepatic Fibrosis using Laser Capture Micro-dissection. We verified that the dysregulated miRNA in CHF whole liver were also dysregulated in diseased cholangiocytes. In silico target prediction of candidate miRNAs shows that molecular pathways that are associated with CHF were under the regulation of dysregulated miRNA targets. These results may provide insight into a regulatory network of miRNAs with crucial functional roles in the disease pathology of CHF.