Branched-chain amino acid (BCAA) metabolic disorders are a group of rare diseases. The disorders are caused by genetic variants that disrupt the metabolic pathways of leucine, isoleucine and valine, and then lead to the accumulation of toxic metabolites. Several disorders are known such as methylmalonic acidemia, maple syrup urine disease, propionic acidemia, mitochondrial short-chain enoyl-CoA hydratase-1 deficiency and 3-hydroxyisobutyryl-CoA hydrolase deficiency. The disorders have complex clinical manifestations and are relatively rare. Their diagnosis mainly relies on metabolic tests and gene analysis. Long-term management is based on dietary intervention and vitamin therapy. In some disorders, early diagnosis and treatment via newborn screening can improve the outcomes. This article systematically reviews the pathogenic genes, molecular mechanisms, clinical phenotypes, metabolic characteristics, and therapeutic advances in BCAA metabolism disorders to provide references for improving precision prevention and management strategies.