In some genetic defects mainly liver cells are affected and leads to liver injury and liver failure. In some genetic defects and inherited metabolic disorders defective gene expression in liver cells leads not to liver disease but to other organ damage due to production abnormal proteins or lack of enzymatic function to clear toxic metabolites. Alpha-one antitrypsin deficiency, Wilson disease, cystic fibrosis, neonatal hemochromatosis, tyrosinemia, galactosemia, organic acidemia, urea cycle defects, fatty acid oxidation defects, glycogen storage diseases, primary hyperoxaluria type 1, liver restricted mitochondrial respiratory chain disorders, Crigler Najjar syndrome, progressive familiyal intrahepatic cholestasis type 1,2,3, protein C deficiency, hemophilia A and B, familial hypercholesterolemia, Alagille syndrome, polycystic liver-kidney disease are such disorders that can be treated with liver transplantation or combined liver-kidney transplantation. Timing, selection, and management of these patients with this rare disorders shows significant improvements. Liver transplantation effectively treats both the underlying defect for those disorders mainly the liver is affected. Inherited diseases that can be treated with liver/liver-kidney transplantation is frequent in Turkey due to high rate of consanguinity. In Baskent University Liver Transplantation Unit, out of 166 pediatric patients with liver transplantation, 54 (32%) had metabolic diseases. 31 (57%) Wilson disease, 7 tyrosinemia, 5 Crigler Najjar disease, 3 glycogen storage disease, 3 primary hyperoxaluria type 1, 2 familial hypercholesterolemia, 1 deoxyguanosine kinase deficiency, 1 3-methyl crotonyl CoA carboxylase deficiency, 1 ornithine transcarbamylase deficiency patients successfully liver transplanted. In addition to 54 metabolic cases, 10 patients with PFIC also underwent liver transplantation in our unit.