ICD-10 Code E80.5 – Crigler-Najjar Syndrome (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code E80.5 – Crigler-Najjar syndrome
What it is
Crigler-Najjar syndrome is a rare inherited disorder of bilirubin metabolism. It causes unconjugated hyperbilirubinemia because the liver cannot properly process bilirubin.
Clinical signs
Typical findings include persistent jaundice beginning in infancy and elevated unconjugated bilirubin. Clinical features vary; refer to documentation.
When to use this code
Use E80.5 when the provider documents Crigler-Najjar syndrome or clearly identifies this inherited bilirubin disorder. It may also apply when the diagnosis is confirmed in the record and the visit addresses the condition itself or its evaluation. Check documentation if the note only mentions jaundice without naming the syndrome.
Do not use for
Do not use this code for neonatal jaundice, physiologic jaundice, or other specified causes of hyperbilirubinemia unless Crigler-Najjar syndrome is documented. Do not assume the diagnosis from bilirubin testing alone.
Coding tip
Code from the provider’s documented diagnosis, not from jaundice history or lab results alone.