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Segmental neurofibromatosis is rare disorder characterized by cafe-au-lait macules and neurofibromas, or only neurofibromas, limited to one region of the body. Typically the neurofibromas are unilateral and are occupied a single dermatome in most patients. We report a 62-year-old man with segmental neurofibromatosis on the right knee in the distribution of dermatomes L3 and L4.