Abstract
Introduction: Segmental neurofibromatosis (NF) is an uncommon mosaic form of neurofibromatosis type 1 (NF1). The majority of cases present early in life (median age 28 years old) and lack the deep involvement classically associated with NF1 including a variety of neural-derived neoplasms. Several subtypes exist including true segmental, localized with deep involvement, hereditary, and bilateral. We report a rare case of localized segmental NF with deep involvement that presented at more than twice the median age of onset.
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