ICD-10-CM 2027
ICD-10 code for HoFH
From the official ICD-10-CM alphabetic index entry “HoFH (homozygous familial hypercholesterolemia)”. Page updated September 29, 2026.
About coding HoFH
The ICD-10-CM code for HoFH is E78.010 (Homozygous familial hypercholesterolemia [HoFH]).
Within E78.01 (familial hypercholesterolemia), E78.010 is specifically for Homozygous familial hypercholesterolemia [HoFH]. Related codes cover Heterozygous familial hypercholesterolemia [HeFH] (E78.011) and unspecified (E78.019).
It is not a CC or MCC, so as a secondary diagnosis it doesn't change the MS-DRG severity level.
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.
Common questions about HoFH ICD-10 codes
What is the ICD-10 code for HoFH?
- E78.010 — Homozygous familial hypercholesterolemia [HoFH].
Is E78.010 billable?
- Yes. E78.010 is a billable/specific ICD-10-CM code valid for FY2027 (October 1, 2026 – September 30, 2027).
Can E78.010 be used as a principal diagnosis?
- Yes. The Medicare Code Editor doesn't restrict E78.010 as a principal diagnosis.
Is E78.010 a CC or MCC?
- No. E78.010 is neither a CC nor an MCC under MS-DRG v44.0.