A 61-year-old female had neurofibromas situated on the right side of upper trunk, shoulder and neck in the distribution of dermatomes C3 and C4(right), which developed 3 years ago. There were not cafe-au-lait spots, axillary frecklings, or Lisch nodule (iris pignented hamartoma). Biopsy specimen showed collagenous wavy fibrils among which spindle-shaped and round cells were embedded as seen in the typical neurofibroma. This case was considered to be a segmental form of von Recklinghausen's disease.