2026 ICD-10-CM Diagnosis Code E03.1Congenital hypothyroidism without goiter

ICD-10-CM Codes›E00–E89›E00-E07›E03

ICD-10-CM E03.1
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

E03.1 is a billable ICD-10-CM diagnosis code for congenital hypothyroidism without goiter. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026) and groups to MS-DRG 643 through 645. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Thyroid disorders.

Code Identity

ICD-10-CM Code
E03.1
Billable Status
Yes — Valid for Submission
Code Describes
Congenital hypothyroidism without goiter
Short Description
Congenital hypothyroidism without goiter
Same as the full description in the CMS dataset.
Parent Code
E03
Other hypothyroidism

Code Classification

ChapterE00–E89Endocrine, nutritional and metabolic diseases
SectionE00-E07Disorders of thyroid gland
CategoryE03Other hypothyroidism
This CodeE03.1Congenital hypothyroidism without goiter

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Brain lung thyroid syndrome
  • Central hypothyroidism
  • Choanal atresia
  • Choanal atresia, athelia, hypothyroidism, delayed puberty, short stature syndrome
  • Congenital absence of thyroid gland
  • Congenital atrophy of thyroid
  • Congenital central hypothyroidism
  • Congenital central hypothyroidism due to thyrotropin-releasing hormone receptor deficiency
  • Congenital hypothyroidism
  • Congenital hypothyroidism due to absence of thyroid gland
  • Congenital hypothyroidism due to congenital anomaly of thyroid gland
  • Congenital hypothyroidism due to dual oxidase maturation factor 2
  • Congenital hypothyroidism due to maternal intake of antithyroid drug
  • Congenital hypothyroidism due to peripheral resistance to thyroid hormone
  • Congenital hypothyroidism due to symporter mutation
  • Congenital hypothyroidism due to thyroglobulin mutation
  • Congenital hypothyroidism due to thyroid deiodinase mutation
  • Congenital hypothyroidism due to thyroid peroxidase mutation
  • Congenital hypothyroidism due to thyroid stimulating hormone receptor mutation
  • Congenital hypothyroidism due to transplacental passage of maternal thyroid stimulating hormone binding inhibitory antibody
  • Congenital hypothyroidism without goiter
  • Congenital iodine deficiency syndrome
  • Congenital thyroid hypoplasia
  • Developmental malformation of branchial arch
  • Ear, face and neck congenital anomalies
  • Familial thyroid dyshormonogenesis
  • Genetic transient congenital hypothyroidism
  • Hypertrophy of testis
  • Hypoplasia of thyroid
  • Hypothyroidism due to mutation in transcription factor of pituitary development
  • Idiopathic congenital hypothyroidism
  • Infant hypothyroidism
  • Neonatal diabetes, congenital hypothyroidism, congenital glaucoma, hepatic fibrosis, polycystic kidney syndrome
  • Neonatal jaundice with congenital hypothyroidism
  • Obesity, colitis, hypothyroidism, cardiac hypertrophy, developmental delay syndrome
  • Primary hypothyroidism
  • Resistance to thyroid hormone due to mutation in thyroid hormone receptor alpha
  • Resistance to thyroid hormone syndrome
  • Thyroid atrophy
  • Thyroid degeneration
  • Thyroid hemiagenesis
  • Transient congenital hypothyroidism due to dual oxidase 2 mutation
  • Transient hypothyroidism
  • X-linked central congenital hypothyroidism with late-onset testicular enlargement

Tabular List NotesGuidance

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

Inclusion Terms

  • Aplasia of thyroid (with myxedema)
  • Congenital atrophy of thyroid
  • Congenital hypothyroidism 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.

    • Absence(of) (organ or part) (complete or partial)
      • thyroid (gland) (acquired)
        • congenital
    • Agenesis
      • thyroid (gland)
    • Aplasia
      • thyroid (congenital) (with myxedema)
    • Athyrea(acquired)
      • congenital
    • Atrophy, atrophic(of)
      • thyroid (gland) (acquired)
        • with cretinism
    • Atrophy, atrophic(of)
      • thyroid (gland) (acquired)
        • congenital (with myxedema)
    • Hypoplasia, hypoplastic
      • thyroid (gland)
    • Hypothyroidism(acquired)
      • congenital (without goiter)
    • Insufficiency, insufficient
      • thyroid (gland) (acquired)
        • congenital
    • Jaundice(yellow)
      • newborn
        • due to or associated with
          • hypothyroidism, congenital
    • Subthyroidism(acquired)
      • congenital

Clinical ClassificationClinical

AHRQ’s CCSR groups this code into broader clinical categories.

CCSR END001
Thyroid disorders
Default principal diagnosis: inpatient Yes · outpatient Yes

Clinical InformationClinical

  • Congenital Hypothyroidism

    a condition in infancy or early childhood due to an in-utero deficiency of thyroid hormones that can be caused by genetic or environmental factors, such as thyroid dysgenesis or hypothyroidism in infants of mothers treated with thiouracil during pregnancy. endemic cretinism is the result of iodine deficiency. clinical symptoms include severe mental retardation, impaired skeletal development, short stature, and myxedema.

  • Choanal Atresia

    a congenital abnormality that is characterized by a blocked choanae, the opening between the nose and the nasopharynx. blockage can be unilateral or bilateral; bony or membranous.

  • Congenital Hypothyroidism

    a deficiency of thyroid hormone present at birth. the etiology can be genetic or environmental, or a combination of both; treatment is based on severity and causality.

  • Congenital Hypothyroidism with Diffuse Goiter|Congenital hypothyroidism with diffuse goiter

    evidence of congenital hypothyroidism with diffuse goiter.

  • Congenital Hypothyroidism with Ectopic Thyroid

    thyroid hormone deficiency present at birth that is associated with ectopic thyroid tissue located in the neck region.

  • Congenital Hypothyroidism without Goiter|Congenital hypothyroidism without goiter

    evidence of congenital hypothyroidism without goiter.

  • Transient Hypothyroxinemia of Prematurity|Hypothyroxinemia of Prematurity|THOP|Transient Congenital Hypothyroidism

    a common, self-limiting thyroid disorder seen in preterm infants that is characterized by abnormally low serum levels of thyroxine and free thyroxine with normal serum levels of thyroid stimulating hormone.

  • Iatrogenic Primary Hypothyroidism

    primary hypothyroidism due to medical or surgical treatment.

  • Primary Hypothyroidism

    abnormally low levels of thyroid hormones due to a disorder originating within the thyroid gland.

  • Transient Primary Hypothyroidism

    primary hypothyroidism that resolves spontaneously.

Patient EducationClinical

Hypothyroidism

Hypothyroidism, or underactive thyroid, happens when your thyroid gland doesn't make enough thyroid hormones to meet your body's needs.

The full article covers:

  • What is hypothyroidism?
  • What causes hypothyroidism?
  • Who is at risk for hypothyroidism?
  • What are the symptoms of hypothyroidism?
  • What other problems can hypothyroidism cause?
  • How is hypothyroidism diagnosed?
  • What are the treatments for hypothyroidism?

Read the full article at MedlinePlus

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

Convert E03.1 to ICD-9-CMHistory

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

ICD-9-CM
243 Congenital hypothyroidsm
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 E03.1Overview

Is E03.1 (Other hypothyroidism) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report congenital hypothyroidism without goiter on HIPAA-covered claims from October 1, 2025 through September 30, 2026.

What MS-DRG does E03.1 group to?

When congenital hypothyroidism without goiter is the principal diagnosis on an inpatient stay, it groups to MS-DRG 643, 644, 645, with relative weights from 0.7683 to 1.6461 depending on complications. Higher weights mean higher Medicare reimbursement.

What is the ICD-9 equivalent of E03.1?

Under the General Equivalence Mappings, congenital hypothyroidism without goiter converts to ICD-9-CM 243 (congenital hypothyroidsm). 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.