2027 ICD-10-CM Diagnosis Code L81.3Cafe au lait spots

ICD-10-CM Codes›L00–L99›L80-L99›L81

ICD-10-CM L81.3
CMSSource: CMS FY 2027 ICD-10-CM dataset · Effective Oct 1, 2026 – Sep 30, 2027

L81.3 is a billable ICD-10-CM diagnosis code for cafe au lait spots. 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, 795. Coders also document this condition as café au lait spots. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Other specified and unspecified skin disorders.

Code Identity

ICD-10-CM Code
L81.3
Billable Status
Yes — Valid for Submission
Code Describes
Cafe au lait spots
Short Description
Cafe au lait spots
Same as the full description in the CMS dataset.
Parent Code
L81
Other disorders of pigmentation

Code Classification

ChapterL00–L99Diseases of the skin and subcutaneous tissue
SectionL80-L99Other disorders of the skin and subcutaneous tissue
CategoryL81Other disorders of pigmentation
This CodeL81.3Cafe au lait spots

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Café au lait spots
  • Café-au-lait macules with pulmonary stenosis
  • Café-au-lait macules with temporal dysrhythmia
  • Intrauterine growth restriction, congenital multiple café au lait macules, increased sister chromatid exchange syndrome
  • Multiple café-au-lait macules due to neurofibromatosis
  • Neurofibromatosis type 1
  • Neurofibromatosis type 6

Instructional NotesGuidance

Instructions from the official ICD-10-CM Tabular List that apply to L81.3: its own notes plus those printed at L81 and Chapter 12. 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 L81 Other disorders of pigmentation applies to 10 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 L81 Other disorders of pigmentation applies to 10 codes
  • nevus - see Alphabetical Index
From Chapter 12 (L00-L99) Diseases of the skin and subcutaneous tissue applies to 1,001 codes
  • certain conditions originating in the perinatal period (P04-P96)
  • certain infectious and parasitic diseases (A00-B99)
  • complications of pregnancy, childbirth and the puerperium (O00-O9A)
  • congenital malformations, deformations, and chromosomal abnormalities (Q00-Q99)
  • endocrine, nutritional and metabolic diseases (E00-E88)
  • lipomelanotic reticulosis (I89.8)
  • neoplasms (C00-D49)
  • symptoms, signs and abnormal clinical and laboratory findings, not elsewhere classified (R00-R94)
  • systemic connective tissue disorders (M30-M36)
  • viral warts (B07.-)

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

Referenced in Other NotesGuidance

Instructional notes printed at other codes that name L81.3, its category, or a range that includes it.

Excludes2 1

These codes carry an Excludes2 note naming L81.3: its condition is not part of those codes, and both may be reported when the patient has both.

  • Q82.5 Congenital non-neoplastic nevus
    Café au lait spots (L81.3)

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 SKN007
Other specified and unspecified skin disorders
Default principal diagnosis: inpatient Yes · outpatient Yes

Clinical InformationClinical

  • Cafe-au-Lait Spots

    light brown pigmented macules associated with neurofibromatosis and albright's syndrome (see fibrous dysplasia, polyostotic).

Patient EducationClinical

Birthmarks

Birthmarks are abnormalities of the skin that are present when a baby is born. There are two types of birthmarks. Vascular birthmarks are made up of blood vessels that haven't formed correctly. They are usually red. Two types of vascular birthmarks are hemangiomas and port-wine stains.

Read the full article at MedlinePlus

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

Convert L81.3 to ICD-9-CMHistory

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

ICD-9-CM
709.09 Other dyschromia
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 L81.3Overview

What is the ICD-10 code for cafe au lait spots?

The ICD-10-CM code for cafe au lait spots is L81.3 (sometimes written as L813). It is billable on HIPAA-covered claims from October 1, 2026 through September 30, 2027.

Is L81.3 (Other disorders of pigmentation) a billable code?

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

What MS-DRG does L81.3 group to?

When cafe au lait spots is the principal diagnosis on an inpatient stay, it groups to MS-DRG 795, 607, 606, with relative weights from 0.2022 to 1.5721 depending on complications. Higher weights mean higher Medicare reimbursement.

Can L81.3 be reported with Q82.5?

Not as a rule. The Excludes1 note at L81, which applies to L81.3, lists "birthmark NOS (Q82.5)". 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).

What is the ICD-9 equivalent of L81.3?

Under the General Equivalence Mappings, cafe au lait spots converts to ICD-9-CM 709.09 (other dyschromia). The mapping is approximate, so confirm the match fits the documentation.