Metabolic liver diseases are a group of disorders that affect the liver primarily or secondarily. Some of the diseases are relatively common. In this study, we are focusing on the update methods for diagnosis and management of these diseases; e.g. the use of: recombinant DNA technology, developmental animal models and basic cell biology for the diagnosis of some diseases and gene therapy for the treatment of some other diseases. Nevertheless, relatively little is known about most metabolic liver diseases or the therapeutic options available to affected children.