---
title: "ICD-10-CM E78.010: Homozygous familial… | BillingBrief"
description: "ICD-10-CM code E78.010: Homozygous familial hypercholesterolemia [HoFH]. Category, related codes, and the official source, in plain English."
canonical: "https://billingbrief.com/icd10/e78.010/"
language: "en"
---

# E78.010 Homozygous familial hypercholesterolemia \[HoFH\]

ICD-10-CM · [CMS code reference](#source)

**Official ICD-10-CM description:** Homozygous familial hypercholesterolemia \[HoFH\]

## Coding notes

From the ICD-10-CM FY2027 (effective October 1, 2026) tabular list. Notes on a parent code or category apply here too.

### Excludes1

Not coded together with this code. The exception is when the two conditions are unrelated to each other.

-   sphingolipidosis (E75.0-E75.3)
-   androgen insensitivity syndrome (E34.5-)
-   congenital adrenal hyperplasia (E25.0)
-   hemolytic anemias attributable to enzyme disorders (D55.-)
-   Marfan syndrome (Q87.4-)
-   5-alpha-reductase deficiency (E29.1)

### Excludes2

Not part of this code, but both can be coded when both are documented.

-   Ehlers-Danlos syndromes (Q79.6-)

## Related codes

-   [`E78.00` Pure hypercholesterolemia, unspecified ICD-10-CM](https://billingbrief.com/icd10/e78.00/)
-   [`E78.011` Heterozygous familial hypercholesterolemia \[HeFH\] ICD-10-CM](https://billingbrief.com/icd10/e78.011/)
-   [`E78.019` Familial hypercholesterolemia, unspecified ICD-10-CM](https://billingbrief.com/icd10/e78.019/)
-   [`E78.1` Pure hyperglyceridemia ICD-10-CM](https://billingbrief.com/icd10/e78.1/)
-   [`E78.2` Mixed hyperlipidemia ICD-10-CM](https://billingbrief.com/icd10/e78.2/)
-   [`E78.3` Hyperchylomicronemia ICD-10-CM](https://billingbrief.com/icd10/e78.3/)
-   [`E78.41` Elevated Lipoprotein(a) ICD-10-CM](https://billingbrief.com/icd10/e78.41/)
-   [`E78.49` Other hyperlipidemia ICD-10-CM](https://billingbrief.com/icd10/e78.49/)

## Common questions

### Is E78.010 a billable code?

Yes. E78.010 is a billable ICD-10-CM code in the ICD-10-CM FY2027 (effective October 1, 2026) code set.

## From the source

Official description from [CMS](https://www.cms.gov/medicare/coding-billing/icd-10-codes).
ICD-10-CM FY2027 (effective October 1, 2026).

Code set

ICD-10-CM

Category

E78 Disorders of lipoprotein metabolism and other lipidemias

Chapter

Endocrine, nutritional and metabolic diseases

Billable

Yes

Educational reference only. Not medical, legal, or billing advice. Always check the current official code set and payer policy before you bill. [About BillingBrief and our sources](https://billingbrief.com/about/).
