Isovaleric acidemia is a disease that prevents the body from breaking down the amino acid leucine. The onset is typically in early infancy and includes symptoms of a distinctive odor, poor feeding, vomiting, seizures, lethargy, organic acidemia, organic aciduria, coma, and premature death. Later onset of the disease is associated with symptoms of failure to thrive and developmental delay. The condition can be managed with a strict diet. The incidence of isovaleric acidemia is estimated to be ~ 1 in 250,000.