2026 ICD-10-CM Diagnosis Code G90.1Familial dysautonomia [Riley-Day]

ICD-10-CM Codes›G00–G99›G89-G99›G90

ICD-10-CM G90.1
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

G90.1 is a billable ICD-10-CM diagnosis code for familial dysautonomia [Riley-Day]. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026). Coders also document this condition as familial dysautonomia. 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
G90.1
Billable Status
Yes — Valid for Submission
Code Describes
Familial dysautonomia [Riley-Day]
Short Description
Familial dysautonomia [Riley-Day]
Same as the full description in the CMS dataset.
Parent Code
G90
Disorders of autonomic nervous system

Code Classification

ChapterG00–G99Diseases of the nervous system
SectionG89-G99Other disorders of the nervous system
CategoryG90Disorders of autonomic nervous system
This CodeG90.1Familial dysautonomia [Riley-Day]

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Familial dysautonomia
  • Hereditary dysautonomia with motor neuropathy

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.

    • Dysautonomia(familial)
    • Riley-Day syndrome

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

Patient EducationClinical

Autonomic Nervous System Disorders

Your autonomic nervous system is the part of your nervous system that controls involuntary actions, such as the beating of your heart and the widening or narrowing of your blood vessels. When something goes wrong in this system, it can cause serious problems, including:

Read the full article at MedlinePlus

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

Convert G90.1 to ICD-9-CMHistory

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

ICD-9-CM
742.8 Nervous system anom NEC
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 G90.1Overview

Is G90.1 (Disorders of autonomic nervous system) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report familial dysautonomia [Riley-Day] on HIPAA-covered claims from October 1, 2025 through September 30, 2026.

What is the ICD-9 equivalent of G90.1?

Under the General Equivalence Mappings, familial dysautonomia [Riley-Day] converts to ICD-9-CM 742.8 (nervous system anom NEC). The mapping is approximate, so confirm the match fits the documentation.

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.