2026 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 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

L81.3 is a billable ICD-10-CM diagnosis code for cafe au lait spots. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026) and groups to MS-DRG 606 through 607. 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

Index to Diseases and InjuriesGuidance

Alphabetical index entries that point to this code.

External Cause of Injuries IndexGuidance

References for this code in the External Cause of Injuries Index.

    • Café, au lait spots
    • Spots, spotting(in) (of)
      • café, au lait

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

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 2017–2025No changes
FY 2026CurrentCurrent code set, no changesEffective October 1, 2025 through September 30, 2026.

Questions About L81.3Overview

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, 2025 through September 30, 2026.

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 606, 607, with relative weights from 0.9064 to 1.5132 depending on complications. Higher weights mean higher Medicare reimbursement.

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.

Footnotes

[1] Not chronic - A diagnosis code that does not fit the criteria for chronic condition (duration, ongoing medical treatment, and limitations) is considered not chronic. Some codes designated as not chronic are acute conditions. Other diagnosis codes that indicate a possible chronic condition, but for which the duration of the illness is not specified in the code description (i.e., we do not know the condition has lasted 12 months or longer) also are considered not chronic.