| E71.0 | Maple-syrup-urine disease | BILLABLE CODE |
| E71.1 | Other disorders of branched-chain amino-acid metabolism | NON-BILLABLE CODE |
| E71.11 | Branched-chain organic acidurias | NON-BILLABLE CODE |
| E71.110 | Isovaleric acidemia | BILLABLE CODE |
| E71.111 | 3-methylglutaconic aciduria | BILLABLE CODE |
| E71.118 | Other branched-chain organic acidurias | BILLABLE CODE |
| E71.12 | Disorders of propionate metabolism | NON-BILLABLE CODE |
| E71.120 | Methylmalonic acidemia | BILLABLE CODE |
| E71.121 | Propionic acidemia | BILLABLE CODE |
| E71.128 | Other disorders of propionate metabolism | BILLABLE CODE |
| E71.19 | Other disorders of branched-chain amino-acid metabolism | BILLABLE CODE |
| E71.2 | Disorder of branched-chain amino-acid metabolism, unspecified | BILLABLE CODE |
| E71.3 | Disorders of fatty-acid metabolism | NON-BILLABLE CODE |
| E71.30 | Disorder of fatty-acid metabolism, unspecified | BILLABLE CODE |
| E71.31 | Disorders of fatty-acid oxidation | NON-BILLABLE CODE |
| E71.310 | Long chain/very long chain acyl CoA dehydrogenase deficiency | BILLABLE CODE |
| E71.311 | Medium chain acyl CoA dehydrogenase deficiency | BILLABLE CODE |
| E71.312 | Short chain acyl CoA dehydrogenase deficiency | BILLABLE CODE |
| E71.313 | Glutaric aciduria type II | BILLABLE CODE |
| E71.314 | Muscle carnitine palmitoyltransferase deficiency | BILLABLE CODE |
| E71.318 | Other disorders of fatty-acid oxidation | BILLABLE CODE |
| E71.32 | Disorders of ketone metabolism | BILLABLE CODE |
| E71.39 | Other disorders of fatty-acid metabolism | BILLABLE CODE |
| E71.4 | Disorders of carnitine metabolism | NON-BILLABLE CODE |
| E71.40 | Disorder of carnitine metabolism, unspecified | BILLABLE CODE |
| E71.41 | Primary carnitine deficiency | BILLABLE CODE |
| E71.42 | Carnitine deficiency due to inborn errors of metabolism | BILLABLE CODE |
| E71.43 | Iatrogenic carnitine deficiency | BILLABLE CODE |
| E71.44 | Other secondary carnitine deficiency | NON-BILLABLE CODE |
| E71.440 | Ruvalcaba-Myhre-Smith syndrome | BILLABLE CODE |
| E71.448 | Other secondary carnitine deficiency | BILLABLE CODE |
| E71.5 | Peroxisomal disorders | NON-BILLABLE CODE |
| E71.50 | Peroxisomal disorder, unspecified | BILLABLE CODE |
| E71.51 | Disorders of peroxisome biogenesis | NON-BILLABLE CODE |
| E71.510 | Zellweger syndrome | BILLABLE CODE |
| E71.511 | Neonatal adrenoleukodystrophy | BILLABLE CODE |
| E71.518 | Other disorders of peroxisome biogenesis | BILLABLE CODE |
| E71.52 | X-linked adrenoleukodystrophy | NON-BILLABLE CODE |
| E71.520 | Childhood cerebral X-linked adrenoleukodystrophy | BILLABLE CODE |
| E71.521 | Adolescent X-linked adrenoleukodystrophy | BILLABLE CODE |
| E71.522 | Adrenomyeloneuropathy | BILLABLE CODE |
| E71.528 | Other X-linked adrenoleukodystrophy | BILLABLE CODE |
| E71.529 | X-linked adrenoleukodystrophy, unspecified type | BILLABLE CODE |
| E71.53 | Other group 2 peroxisomal disorders | BILLABLE CODE |
| E71.54 | Other peroxisomal disorders | NON-BILLABLE CODE |
| E71.540 | Rhizomelic chondrodysplasia punctata | BILLABLE CODE |
| E71.541 | Zellweger-like syndrome | BILLABLE CODE |
| E71.542 | Other group 3 peroxisomal disorders | BILLABLE CODE |
| E71.548 | Other peroxisomal disorders | BILLABLE CODE |
