1-To review the classification of inborn errors of metabolism.2- To review the pathophysiology basic principles of the inborn errors of metabolism.3-To illustrate imaging findings related to genetically determined metabolic disorders.

1 - Introduction2 - Classification of inborn errors of metabolismDisorders of intermediary metabolism : aminoacidopathies, organic acidurias, latic acidemias, hyperammonemia and disorders of the urea cycle, disorders of fatty acid oxidation. Disorders of the biosynthesis and breakdown of complex molecules: purine and pyrimidine metabolism, lipoprotein metabolism, lysosomal storage disorders, peroxisomal disorders, porphyrias.Mitochondrial disorders: mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes (MELAS), Kearns-Sayre syndrome, MERRF (myoclonus epilepsy with ragged red fibers). Neurotransmitter defects and related disorders: disorders of glycine and serine metabolism.Disorders of mineral metabolism: Menkes disease, Wilson disease, others.3- General and specific clinical features of inborn errors of metabolism4 - Clinico-radiological approach to inborn errors of metabolism with illustrative cases5- Take home message6 - Conclusion7 - References