A 71-year-old woman presented with multiple, flesh-colored, papular lesions on the left dorsal side of the hand which had erupted 5 years earlier. No cafè-au-lait spots, freckles, or Lisch nodules were detected. Family history did not disclose neurofibromas or abnormal pigmentation. Two biopsy specimens of the lesions showed circumscribed, non-encapsulated neurofibromas. Segmental neurofibromatosis is characterized by the unilateral, segmental appearance of neurofibromas and/or cafè-au-lait spots in the absence of genetic transmission. The authors discuss the significance of localized multiple cutaneous neurofibromas in the absence of family history and suggest the possibility of a cutaneous hamartoma.