Segmental neurofibromatosis (NF) is characterized by the strictly unilateral occurrence of features that are typical of the more ordinary forms of NF (i.e., NF-1, NF-2), including schwannomas, cutaneous or plexiform neurofibromas, and/or café au lait spots. That is, these features are found in only one or several dermal segments. We describe a case of a 68-year-old woman affected by neurofibromas restricted to the right lumbar region. After extensive investigations, including magnetic resonance imaging, we could rule out the presence of additional, more widespread lesions. Previous reports of segmental NF are reviewed.