ICD-10 Code E76.01 – Hurler’s syndrome (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code E76.01 – Hurler’s syndrome
What it is
E76.01 identifies Hurler’s syndrome, a severe inherited lysosomal storage disorder in the mucopolysaccharidosis I spectrum. It reflects deficiency of alpha-L-iduronidase causing buildup of glycosaminoglycans.
Clinical signs
Typical findings include coarse facial features, developmental delay, skeletal abnormalities, corneal clouding, and organ enlargement. Clinical features vary; refer to documentation.
When to use this code
Use E76.01 when the provider documents Hurler’s syndrome or MPS I, Hurler type. Apply it for confirmed diagnoses in inpatient, outpatient, or problem-list documentation when the record clearly identifies this specific subtype.
Do not code it from family history, carrier status, or a nonspecific mucopolysaccharidosis note. Check documentation if the chart only says MPS I without subtype.
Do not use for
Do not use this code for other mucopolysaccharidoses, Hurler-Scheie syndrome, or Scheie syndrome. If the documentation is nonspecific or unclear, check documentation.
Coding tip
Confirm the exact MPS I subtype in the provider note before assigning E76.01.