Q85.01 Neurofibromatosis, type 1

Billable ICD-10-CM code, FY2026. Exempt from present-on-admission (POA) reporting.

Applicable to

• Von Recklinghausen disease

Applies from Q85 Phakomatoses, not elsewhere classified

Excludes1 (never code together)

• ataxia telangiectasia [Louis-Bar] (G11.3)
• familial dysautonomia [Riley-Day] (G90.1)

Risk adjustment (HCC)

• CMS-HCC V28: HCC 23 — Prostate, Breast, and Other Cancers and Tumors
• CMS-HCC V22: HCC 12 — Breast, Prostate, and Other Cancers and Tumors
• ESRD V24: HCC 12

MS-DRG index

Defines the logic of DRG(s) 091-093 in MDC 01.

Approximate ICD-9-CM (GEMs)

• 237.71 Neurofibromatosis, type 1 [von recklinghausen's disease]

Code annotations containing back-references to Q85.01

Back-references to Q85.0

• Type 1 Excludes: D33 Benign neoplasm of brain and other parts of central nervous system ("neurofibromatosis (Q85.0-)")
• Type 1 Excludes: D36.1 Benign neoplasm of peripheral nerves and autonomic nervous system ("neurofibromatosis (Q85.0-)")
• Type 1 Excludes: D48 Neoplasm of uncertain behavior of other and unspecified sites ("neurofibromatosis (nonmalignant) (Q85.0-)")
• Type 2 Excludes: Q07 Other congenital malformations of nervous system ("neurofibromatosis (nonmalignant) (Q85.0-)")

Diagnosis Index entries for Q85.01

• Neurofibromatosis (multiple) (nonmalignant) › type 1 (von Recklinghausen)
• Recklinghausen disease
• Von Recklinghausen › disease (neurofibromatosis)

Nearby codes

• Q85 Phakomatoses, not elsewhere classified
• Q85.0 Neurofibromatosis (nonmalignant)
• Q85.00 Neurofibromatosis, unspecified
• Q85.01 Neurofibromatosis, type 1 (this code)
• Q85.02 Neurofibromatosis, type 2
• Q85.03 Schwannomatosis
• Q85.09 Other neurofibromatosis
• Q85.1 Tuberous sclerosis
• Q85.8 Other phakomatoses, not elsewhere classified