Excludes1 pair

E25.0 vs Q89.1

E25.0 and Q89.1 are not coded together. ICD-10-CM declares them mutually exclusive with an Excludes1 note — the two conditions are not expected to occur together, so exactly one of them is normally correct for a given encounter. The Official Guidelines carve out one exception: if the two conditions are clearly unrelated to each other, both may be reported.

The official note, declared on Q89.1
congenital adrenal hyperplasia (E25.0)
E25.0

Congenital adrenogenital disorders associated with enzyme deficiency

Billable
Chapter 4
Endocrine, nutritional and metabolic diseases
Section
E20-E35
Use when the record says
  • Congenital adrenal hyperplasia
  • 21-Hydroxylase deficiency
  • Salt-losing congenital adrenal hyperplasia
Indexed under
  • Adrenogenitalism, congenital
  • Adrenogenital syndrome › congenital
  • Adrenogenital syndrome › salt loss
  • Defect, defective › 3-beta-hydroxysteroid dehydrogenase
  • Defect, defective › 11-hydroxylase
Q89.1

Congenital malformations of adrenal gland

Billable
Chapter 17
Congenital malformations, deformations, chromosomal abnormalities, and genetic disorders
Section
Q80-Q89
Indexed under
  • Aberrant › adrenal gland
  • Absence › adrenal (gland) (congenital)
  • Accessory › adrenal gland
  • Agenesis › adrenal (gland)
  • Anomaly, anomalous › adrenal (gland)

These two codes sit in different chapters of the Tabular List, which is usually the clearest signal that they describe different underlying processes rather than different degrees of the same one.

Excludes1 is not always absolute: where two conditions covered by the note are genuinely unrelated, CMS guidance permits reporting both. Document the reasoning rather than assuming it. More on Excludes1 vs Excludes2 · FY2027