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ICD-10-CM 2027 diagnosis code

E70.0Classical phenylketonuria

✓ Billable / specificCC — complication/comorbidity

E70.0 is a valid, billable ICD-10-CM code for classical phenylketonuria. It can be reported on claims for dates of service from October 1, 2026 – September 30, 2027.

Looking up by condition? See ICD-10 code for Følling's disease.

Code unchanged since ICD-10-CM took effect on October 1, 2015. Source: official FY2027 ICD-10-CM release from CDC/NCHS and CMS. Page updated September 29, 2026. About our data

About E70.0

E70.0 is the ICD-10-CM diagnosis code for classical phenylketonuria. It belongs to category E70 (disorders of aromatic amino-acid metabolism), block E70-E88 (metabolic disorders) and chapter 4 (endocrine, nutritional and metabolic diseases). It is billable, so it can be reported on its own for dates of service from October 1, 2026 through September 30, 2027.

Within E70 (disorders of aromatic amino-acid metabolism), E70.0 is specifically for classical phenylketonuria. Related codes cover other hyperphenylalaninemias (E70.1), disorders of tyrosine metabolism (E70.2), albinism (E70.3), disorders of histidine metabolism (E70.4), disorders of tryptophan metabolism (E70.5), other disorders of aromatic amino-acid metabolism (E70.8) and disorder of aromatic amino-acid metabolism, unspecified (E70.9).

As a secondary diagnosis it is a CC (complication or comorbidity), which can move an inpatient stay into the "with CC" MS-DRG of its family, unless the principal diagnosis excludes it.

In MS-DRG v44.0, it is part of the grouping logic for DRG 642 (Inborn and Other Disorders of Metabolism, relative weight 1.3219), in MDC 10 (Endocrine, Nutritional and Metabolic Diseases and Disorders), as a principal or secondary diagnosis. The DRG a claim lands in depends on the principal diagnosis, procedures and any CC/MCC secondary diagnoses.

It has been part of ICD-10-CM since the code set took effect on October 1, 2015 and hasn't changed since.

Before ICD-10, this condition was coded in ICD-9-CM as 270.1 (phenylketonuria [PKU]).

Notes that apply from higher levels

Instructions written at a parent level also apply to E70.0.

Broader instructions also apply from E70-E88 Metabolic disorders and Chapter 4: Endocrine, nutritional and metabolic diseases.

Alphabetic index entries

4 entries

Terms in the official ICD-10-CM index that lead to E70.0.

MS-DRG v44.0 grouping

All DRGs →

Inpatient MS-DRGs this diagnosis can group to, as the principal or a secondary diagnosis, depending on the rest of the claim.

MDC 10 · Endocrine, Nutritional and Metabolic Diseases and Disorders

ICD-9-CM equivalent

Converter →
  • 270.1Phenylketonuria [PKU]approximate

From the CMS 2018 General Equivalence Mappings (GEMs), the final GEMs release.

Code history

  1. 2016
  2. 2017
  3. 2018
  4. 2019
  5. 2020
  6. 2021
  7. 2022
  8. 2023
  9. 2024
  10. 2025
  11. 2026
  12. 2027
  • FY2016 (effective 10/1/2015): Added (first year of ICD-10-CM)
  • No changes since FY2016.

Common questions about E70.0

Is E70.0 billable?

Yes. E70.0 is a billable/specific ICD-10-CM code valid for FY2027 (October 1, 2026 – September 30, 2027).

Can E70.0 be used as a principal diagnosis?

Yes. The Medicare Code Editor doesn't restrict E70.0 as a principal diagnosis.

Is E70.0 a CC or MCC?

E70.0 is a CC (complication or comorbidity) under MS-DRG v44.0, unless excluded by the principal diagnosis.

What DRG does E70.0 group to?

E70.0 is used in the MS-DRG v44.0 logic for MS-DRG 642 (Inborn and Other Disorders of Metabolism). Which one applies depends on whether it is the principal diagnosis and on the rest of the claim.

What is the ICD-9 code for E70.0?

The CMS General Equivalence Mappings map E70.0 to ICD-9-CM 270.1.