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A case of unilateral parapharyngeal neurofibroma occurring in a 25 year old female with von Recklinghausen’s disease is discussed in this report because of the rarity of such a condition. Parapharyngeal neurofibroma usually arises from the vagus nerve and very rarely they are associated with von Recklinghausen’s disease or Neurofibroamatosis type 1. It typically presents with a long–standing history of throat/neck mass with/without neurological deficit. CT scan reveals a moderately enhancing mass, occasionally with central necrosis or cavitations. This, together with spindle shaped cells seen on aspiration cytology, if done, strongly suggests a neurofibroma and excision of the tumor is the treatment of choice. In this patient no sign of malignancy was detected and the two year follow–up period was uneventful.
