---
title: "ICD-10-CM E78.71: Barth syndrome | BillingBrief"
description: "ICD-10-CM code E78.71: Barth syndrome. Category, related codes, and the official source, in plain English."
canonical: "https://billingbrief.com/icd10/e78.71/"
language: "en"
---

# E78.71 Barth syndrome

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

**Official ICD-10-CM description:** Barth syndrome

## 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.

-   Niemann-Pick disease type C (E75.242)
-   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

-   [`E78.49` Other hyperlipidemia ICD-10-CM](https://billingbrief.com/icd10/e78.49/)
-   [`E78.5` Hyperlipidemia, unspecified ICD-10-CM](https://billingbrief.com/icd10/e78.5/)
-   [`E78.6` Lipoprotein deficiency ICD-10-CM](https://billingbrief.com/icd10/e78.6/)
-   [`E78.70` Disorder of bile acid and cholesterol metabolism, unspecified ICD-10-CM](https://billingbrief.com/icd10/e78.70/)
-   [`E78.72` Smith-Lemli-Opitz syndrome ICD-10-CM](https://billingbrief.com/icd10/e78.72/)
-   [`E78.79` Other disorders of bile acid and cholesterol metabolism ICD-10-CM](https://billingbrief.com/icd10/e78.79/)
-   [`E78.81` Lipoid dermatoarthritis ICD-10-CM](https://billingbrief.com/icd10/e78.81/)
-   [`E78.89` Other lipoprotein metabolism disorders ICD-10-CM](https://billingbrief.com/icd10/e78.89/)

## Common questions

### Is E78.71 a billable code?

Yes. E78.71 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/).