2026 ICD-10-CM Diagnosis Code E78.019Familial hypercholesterolemia, unspecified

ICD-10-CM Codes›E00–E89›E70-E88›E78

ICD-10-CM E78.019
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

E78.019 is a billable ICD-10-CM diagnosis code for familial hypercholesterolemia, unspecified. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026). Coders also document this condition as familial defective apolipoprotein B-100.

Code Identity

ICD-10-CM Code
E78.019
Billable Status
Yes — Valid for Submission
Code Describes
Familial hypercholesterolemia, unspecified
Short Description
Familial hypercholesterolemia, unspecified
Same as the full description in the CMS dataset.
Parent Code
E78.01
Familial hypercholesterolemia

Code Classification

ChapterE00–E89Endocrine, nutritional and metabolic diseases
SectionE70-E88Metabolic disorders
CategoryE78Disorders of lipoprotein metabolism and other lipidemias
This CodeE78.019Familial hypercholesterolemia, unspecified

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Familial defective apolipoprotein B-100
  • Familial hyperalphalipoproteinemia
  • Familial hypercholesterolemia
  • Familial hypercholesterolemia due to genetic defect of apolipoprotein B
  • Hyperalphalipoproteinemia
  • Hypercholesterolemia due to cholesterol 7alpha-hydroxylase deficiency
  • Synthetic defect of bile acids

Tabular List NotesGuidance

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

Inclusion Terms

  • Familial hypercholesterolemia NOS

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.

    • Cholesterolemia(essential) (pure)
      • familial
    • Cholesterolemia(essential) (pure)
      • hereditary
    • Hypercholesterolemia(essential) (primary) (pure)
      • familial
    • Hypercholesterolemia(essential) (primary) (pure)
      • hereditary

Code History & ChangesHistory

New Code E78.019 was added to the ICD-10-CM code set for FY 2026, effective October 1, 2025.

Replacement E78.019 replaces the following previously assigned code(s):

  • E78.01 - Familial hypercholesterolemia
FY 2026AddedAdded to the ICD-10-CM code setEffective October 1, 2025.
FY 2026CurrentRevised in the current code setEffective October 1, 2025 through September 30, 2026.

Questions About E78.019Overview

Is E78.019 (Familial hypercholesterolemia) a billable code?

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