2027 ICD-10-CM Diagnosis Code Q80.4Harlequin fetus

ICD-10-CM Codes›Q00-Q99›Q80-Q89›Q80

ICD-10-CM Q80.4
CMSSource: CMS FY 2027 ICD-10-CM dataset · Effective Oct 1, 2026 – Sep 30, 2027

Q80.4 is a billable ICD-10-CM diagnosis code for harlequin fetus. It is valid on HIPAA claims for fiscal year 2027 (October 1, 2026 through September 30, 2027) and groups to MS-DRG 606 through 607. The code is exempt from POA reporting. Coders also document this condition as congenital ectropion. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Other specified and unspecified congenital anomalies.

Code Identity

ICD-10-CM Code
Q80.4
Billable Status
Yes — Valid for Submission
Code Describes
Harlequin fetus
Short Description
Harlequin fetus
Same as the full description in the CMS dataset.
Parent Code
Q80
Congenital ichthyosis

Code Classification

ChapterQ00-Q99Congenital malformations, deformations and chromosomal abnormalities
SectionQ80-Q89Other congenital malformations
CategoryQ80Congenital ichthyosis
This CodeQ80.4Harlequin fetus

Present on Admission (POA)Billing

Q80.4 is exempt from POA reporting on inpatient claims to general acute care hospitals. Review the other POA exempt codes in Other congenital malformations (Q80-Q89).

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Congenital ectropion
  • Congenital ectropion of lip
  • Harlequin change
  • Harlequin fetus
  • Harlequin ichthyosis
  • Harlequin syndrome
  • Segmental hyperhidrosis

Instructional NotesGuidance

Instructions from the official ICD-10-CM Tabular List that apply to Q80.4: its own notes plus those printed at Q80 and Chapter 17. A note printed at a category, block or chapter applies to every code under it.

Excludes1

Not coded here: the excluded code is never reported together with this one, unless the two conditions are unrelated.

From Q80 Congenital ichthyosis applies to 7 codes

Excludes2

Not included here: the excluded condition is not part of this code, but a patient may have both, so both codes may be reported.

From Chapter 17 (Q00-QA1) Congenital malformations, deformations and chromosomal abnormalities applies to 900 codes
  • inborn errors of metabolism (E70-E88)

Notes

General instructions on how these codes are used.

From Chapter 17 (Q00-QA1) Congenital malformations, deformations and chromosomal abnormalities applies to 900 codes
  • Codes from this chapter are not for use on maternal records

Source: CMS ICD-10-CM Tabular List. How to read instructional notes.

Referenced in Other NotesGuidance

Instructional notes printed at other codes that name Q80.4, its category, or a range that includes it.

Excludes1 2

These codes carry an Excludes1 note naming Q80.4: they are not reported together with it, unless the two conditions are unrelated.

  • L85.0 Acquired ichthyosis
    congenital ichthyosis (Q80.-) names Q80.-, which includes this code
  • P83 Other conditions of integument specific to newborn
    congenital malformations of skin and integument (Q80-Q84) names Q80-Q84, which includes this code

Index to Diseases and InjuriesGuidance

Alphabetical index entries that point to this code.

Clinical ClassificationClinical

AHRQ’s CCSR groups this code into broader clinical categories.

CCSR MAL010
Other specified and unspecified congenital anomalies
Default principal diagnosis: inpatient Yes · outpatient Yes

Patient EducationClinical

Skin Conditions

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Read the full article at MedlinePlus

Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.

Convert Q80.4 to ICD-9-CMHistory

The closest ICD-9-CM equivalents under the General Equivalence Mappings.

ICD-9-CM
757.1 Ichthyosis congenita
Approximate The match is approximate rather than exact.

Code HistoryHistory

FY 2016AddedAdded to the ICD-10-CM code setEffective October 1, 2015, the first year of ICD-10-CM.
FY 2017–2026No changes
FY 2027CurrentCurrent code set, no changesEffective October 1, 2026 through September 30, 2027.

Questions About Q80.4Overview

What is the ICD-10 code for harlequin fetus?

The ICD-10-CM code for harlequin fetus is Q80.4 (sometimes written as Q804). It is billable on HIPAA-covered claims from October 1, 2026 through September 30, 2027.

Is Q80.4 (Congenital ichthyosis) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report harlequin fetus on HIPAA-covered claims from October 1, 2026 through September 30, 2027.

What MS-DRG does Q80.4 group to?

When harlequin fetus is the principal diagnosis on an inpatient stay, it groups to MS-DRG 607, 606, with relative weights from 0.8772 to 1.5721 depending on complications. Higher weights mean higher Medicare reimbursement.

Is Q80.4 exempt from POA reporting?

Yes. CMS lists this code among those exempt from present on admission reporting, so hospitals do not assign a POA indicator for harlequin fetus on inpatient claims. The code appears in the Other congenital malformations (Q80-Q89) range of the CMS exempt list.

Can Q80.4 be reported with G60.1?

Not as a rule. The Excludes1 note at Q80, which applies to Q80.4, lists "Refsum's disease (G60.1)". An Excludes1 note means the excluded code is never reported together with this one; the only exception is when the two conditions are unrelated to each other (Official Guidelines, Section I.A.12.a).