FY2027
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Excludes1 pair

Q56- vs Q99.1

Q56 and Q99.1 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 Q56
46,XX true hermaphrodite (Q99.1)
Q56

Indeterminate sex and pseudohermaphroditism

Not billable
Chapter 17
Congenital malformations, deformations, chromosomal abnormalities, and genetic disorders
Section
Q50-Q56
Q99.1

46, XX true hermaphrodite

Billable
Chapter 17
Congenital malformations, deformations, chromosomal abnormalities, and genetic disorders
Section
Q90-Q99
Use when the record says
  • 46, XX with streak gonads
  • 46, XY with streak gonads
  • Pure gonadal dysgenesis
Indexed under
  • Swyer syndrome
  • Hermaphrodite, hermaphroditism › 46,XX with streak gonads
  • Hermaphrodite, hermaphroditism › 46,XY with streak gonads
  • Syndrome › Swyer
  • Dysgenesis › gonadal › pure

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