ICD-10 Code E78.01 – Familial hypercholesterolemia (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code E78.01 – Familial hypercholesterolemia
What it is
Familial hypercholesterolemia is an inherited disorder that causes abnormally high LDL cholesterol from birth. Use this code when the record identifies the condition as familial or hereditary hypercholesterolemia.
Clinical signs
Clinical features vary; refer to documentation. Common references describe markedly elevated LDL cholesterol, premature atherosclerotic disease, and sometimes tendon xanthomas or a family history of early cardiovascular disease.
When to use this code
Use E78.01 when the provider documents familial hypercholesterolemia, FH, or a genetic form of high cholesterol. It also fits encounters focused on evaluation, monitoring, or follow-up of the disorder. Check documentation if the note only says hyperlipidemia or mixed dyslipidemia.
Do not use for
Do not use this code for routine hypercholesterolemia without a familial or hereditary basis. If the record supports a different lipid disorder or the diagnosis is unspecified, code the documented condition instead.
Coding tip
Look for explicit wording such as “familial,” “heterozygous,” “homozygous,” or “FH” before assigning E78.01.