2026 ICD-10-CM Diagnosis Code G11.11Friedreich ataxia

ICD-10-CM Codes›G00–G99›G10-G14›G11

ICD-10-CM G11.11
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

G11.11 is a billable ICD-10-CM diagnosis code for friedreich ataxia. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026). Coders also document this condition as cardiomyopathy in Friedreich's ataxia. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Other nervous system disorders (often hereditary or degenerative).

Code Identity

ICD-10-CM Code
G11.11
Billable Status
Yes — Valid for Submission
Code Describes
Friedreich ataxia
Short Description
Friedreich ataxia
Same as the full description in the CMS dataset.
Parent Code
G11.1
Early-onset cerebellar ataxia

Code Classification

ChapterG00–G99Diseases of the nervous system
SectionG10-G14Systemic atrophies primarily affecting the central nervous system
CategoryG11Hereditary ataxia
This CodeG11.11Friedreich ataxia

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Cardiomyopathy in Friedreich's ataxia
  • Cerebellar ataxia associated with another disorder
  • Dilated cardiomyopathy due to Friedreich's ataxia
  • Friedreich ataxia
  • Hypertrophic cardiomyopathy due to Friedreich ataxia
  • Posterior cord syndrome due to Friedreich ataxia

Tabular List NotesGuidance

Coding notes and annotation back-references applicable to this code.

Inclusion Terms

  • Autosomal recessive Friedreich ataxia
  • Friedreich ataxia with retained reflexes

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.

    • Ataxia, ataxy, ataxic
      • autosomal recessive Friedreich
    • Ataxia, ataxy, ataxic
      • Friedreich's (heredofamilial) (cerebellar) (spinal) (with retained reflexes)
    • Ataxia, ataxy, ataxic
      • hereditary
        • spinal (Friedreich's)
    • Ataxia, ataxy, ataxic
      • spinal
        • hereditary (Friedreich's)
    • Cardiomyopathy(familial) (idiopathic)
      • due to
        • Friedreich's ataxia
    • Disease, diseased
      • Friedreich's
        • combined systemic or ataxia
    • Friedreich's
      • ataxia
    • Friedreich's
      • combined systemic disease
    • Friedreich's
      • sclerosis (cerebellum) (spinal cord)
    • Myocardiopathy(congestive) (constrictive) (familial) (hypertrophic nonobstructive) (idiopathic) (infiltrative) (obstructive) (primary) (restrictive) (sporadic)
      • in (due to)
        • Friedreich's ataxia
    • Sclerosis, sclerotic
      • Friedreich's (spinal cord)
    • Sclerosis, sclerotic
      • hereditary
        • spinal (Friedreich's ataxia)
    • Sclerosis, sclerotic
      • spinal (cord) (progressive)
        • hereditary (Friedreich's) (mixed form)

Clinical ClassificationClinical

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

CCSR NVS006
Other nervous system disorders (often hereditary or degenerative)
Default principal diagnosis: inpatient Yes · outpatient Yes

Clinical InformationClinical

  • Frataxin

    a protein involved in the transfer of iron and sulfur to iron-sulfur cluster (isc) assembly complex for de novo synthesis of a [2fe-2s] cluster, the first step of the mitochondrial iron-sulfur protein biogenesis. deficiency leads to the neurodegenerative disease friedreich ataxia.

  • Friedreich Ataxia

    an autosomal recessive disease, usually of childhood onset, characterized pathologically by degeneration of the spinocerebellar tracts, posterior columns, and to a lesser extent the corticospinal tracts. clinical manifestations include gait ataxia, pes cavus, speech impairment, lateral curvature of spine, rhythmic head tremor, kyphoscoliosis, congestive heart failure (secondary to a cardiomyopathy), and lower extremity weakness. most forms of this condition are associated with a mutation in a gene on chromosome 9, at band q13, which codes for the mitochondrial protein frataxin. (from adams et al., principles of neurology, 6th ed, p1081; n engl j med 1996 oct 17;335(16):1169-75) the severity of friedreich ataxia associated with expansion of gaa repeats in the first intron of the frataxin gene correlates with the number of trinucleotide repeats. (from durr et al, n engl j med 1996 oct 17;335(16):1169-75)

Patient EducationClinical

Friedreich Ataxia

Friedreich ataxia is an inherited disease that damages your nervous system. The damage affects your spinal cord and the nerves that control muscle movement in your arms and legs. Symptoms usually begin between the ages of 5 and 15. The main symptom is ataxia, which means trouble coordinating movements. Specific symptoms include:

Read the full article at MedlinePlus

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

Code History & ChangesHistory

Replacement G11.11 replaces the following previously assigned code(s):

  • G11.1 - Early-onset cerebellar ataxia
FY 2021AddedAdded to the ICD-10-CM code setEffective October 1, 2020.
FY 2022–2025No changes
FY 2026CurrentCurrent code set, no changesEffective October 1, 2025 through September 30, 2026.

Questions About G11.11Overview

Is G11.11 (Early-onset cerebellar ataxia) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report friedreich ataxia on HIPAA-covered claims from October 1, 2025 through September 30, 2026.

Footnotes

[1] Chronic - a chronic condition code indicates a condition lasting 12 months or longer and its effect on the patient based on one or both of the following criteria:

  • The condition results in the need for ongoing intervention with medical products,treatment, services, and special equipment
  • The condition places limitations on self-care, independent living, and social interactions.