Q85 Phakomatoses, not elsewhere classified

Category / Non-billable header 13 subcodes

Block: Other congenital malformations (Q80-Q89)

Excludes 1 (cannot be coded together)

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

Subcodes

Subcodes are listed below for reference. Detail pages are not available for chapters 11–22.

Code Description Billable
Q85..0 Neurofibromatosis (nonmalignant) No
Q85..00 Neurofibromatosis, unspecified Yes
Q85..01 Neurofibromatosis, type 1 Yes
Q85..02 Neurofibromatosis, type 2 Yes
Q85..03 Schwannomatosis Yes
Q85..09 Other neurofibromatosis Yes
Q85..1 Tuberous sclerosis Yes
Q85..8 Other phakomatoses, not elsewhere classified No
Q85..81 PTEN hamartoma tumor syndrome Yes
Q85..82 Other Cowden syndrome Yes
Q85..83 Von Hippel-Lindau syndrome Yes
Q85..89 Other phakomatoses, not elsewhere classified Yes
Q85..9 Phakomatosis, unspecified Yes

Last reviewed: September 2026