Segmental neurofibromatosis is a rare condition that most commonly affects women and presents as neurofibromas, café au lait spots, and freckling. The neurofibromas usually occur in a dermatomal pattern, whereas the café au lait spots may follow Blaschko’s lines. There is often no family history. The etiology is due to a postzygotic mutation in the gene encoding neurofibromin. Histopathology reveals a tumor with haphazardly arranged spindle cells and a pale myxoid stroma from mucin deposition and fibroplasia. There have been few reported cases of segmental neurofibromatosis. In this case report, we will discuss the clinical features, histopathology, differential diagnosis, and management of segmental neurofibromatosis.