ICD-10-CM 2027
ICD-10 code for Klippel's disease
From the official ICD-10-CM alphabetic index entry “Klippel's disease”. Page updated September 29, 2026.
About coding Klippel's disease
The ICD-10-CM code for Klippel's disease is I67.2 (Cerebral atherosclerosis).
Within I67 (other cerebrovascular diseases), I67.2 is specifically for cerebral atherosclerosis. Related codes cover dissection of cerebral arteries, nonruptured (I67.0), cerebral aneurysm, nonruptured (I67.1), progressive vascular leukoencephalopathy (I67.3), hypertensive encephalopathy (I67.4), Moyamoya disease (I67.5), nonpyogenic thrombosis of intracranial venous system (I67.6), cerebral arteritis, not elsewhere classified (I67.7), other specified cerebrovascular diseases (I67.8) and 1 more.
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 070 (Other Cerebrovascular Disorders with MCC, relative weight 1.6093), DRG 071 (Other Cerebrovascular Disorders with CC, relative weight 1.0177) and DRG 072 (Other Cerebrovascular Disorders without CC/MCC, relative weight 0.7348), in MDC 01 (Diseases and Disorders of the Nervous System), 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 Klippel's disease ICD-10 codes
What is the ICD-10 code for Klippel's disease?
- I67.2 — Cerebral atherosclerosis.
Is I67.2 billable?
- Yes. I67.2 is a billable/specific ICD-10-CM code valid for FY2027 (October 1, 2026 – September 30, 2027).
Can I67.2 be used as a principal diagnosis?
- Yes. The Medicare Code Editor doesn't restrict I67.2 as a principal diagnosis.
Is I67.2 a CC or MCC?
- No. I67.2 is neither a CC nor an MCC under MS-DRG v44.0.