Isovaleric acidemia is an inherited disorder in which the body cannot properly process a particular amino acid called leucine due to defects in the enzyme isovaleryl-CoA dehydrogenase (IVD). One unique sign of isovaleric acidemia is a sweaty-foot odor coming from the body of an affected person. The more severe form has an onset within the first few days of life with symptoms that include poor feeding, vomiting, weakness, which eventually progress to seizures, coma and possibly death. Some individuals with isovaleric acidemia don't show symptoms until one year of age or later in childhood. Symptoms come and go, a situation referred to as a metabolic crisis, brought about by illness, infection, periods of fasting or eating large amounts of protein. If not carefully controlled, repeated episodes of these metabolic crises can cause brain damage, resulting in mild to moderate intellectual disability.The child may appear to be normal between these episodes, although they may have poor growth or muscle weakness. Long-term outcome can be excellent if appropriately managed with a special diet and medication. Isovaleric acidemia is caused by pathogenic variants in the IVD gene.