This chapter describes ductal plate malformation-related hereditary liver diseases. Ductal plate malformation (DPM)-related hereditary liver diseases are also called as fibropolycystic diseases of liver. DPM-related hereditary liver diseases include congenital hepatic fibrosis, autosomal recessive polycystic disease, autosomal dominant polycystic disease, Caroli’s disease, and choledochal cysts. This chapter covers the congenital hepatic fibrosis, Caroli’s syndrome, Caroli’s disease, autosomal dominant polycystic disease, choledochal cyst, Von meyenberg complex or biliary microhamartoma, and liver biopsy sign out. CHF are often misdiagnosed as cirrhosis radiologically. Biopsy features of absence of inflammation, single cell plate thick hepatocyte cords, and absent portal vein profiles in most tracts help to differentiate from cirrhosis.