<h4>Background</h4>Isovaleric acidaemia (IVA), characterized by an acute metabolic crisis and psychomotor delay, is a rare inherited metabolic disease caused by a deficiency in isovaleryl-CoA dehydrogenase (IVD).<h4>Methods</h4>We report the case of a Chinese patient with IVA who was admitted to Tianjin Children's Hospital and followed up for 8 years. The gene discussed is IVD; the disease is metabolic disease.