This review is focused on current findings implicating miRNAs in the polycystic liver diseases, which we categorized as cholangiociliopathies. Our recent data suggest that deregulation of miRNA pathways is emerging as a novel mechanism in the development of cholangiociliopathies. Experimental evidence demonstrates that miRNAs (i.e., miR-15a) influence hepatic cyst growth by affecting the expression of the cell cycle regulator, Cdc25A. Given that abnormalities in many cellular processes (i.e., cell cycle regulation, cell proliferation, cAMP and calcium signaling, the EGF-stimulated mitogen-activated protein kinase (MAPK) pathway and fluid secretion) contribute to the hepatic cystogenesis, the potential role of miRNAs in regulation of these processes is discussed.