Neurofibromatosis (NF) or von Recklinghausen's disease is frequently accompanied by malignant tumours, which can occur at any site in the body. These malignancies are mainly of soft tissue origin. Of all head and neck malignancies, the number of soft tissue sarcomas is limited and in combination with NF this type of tumour is a rare event. In this report we describe the clinical course of a young female patient with NP, who presented with a massive malignant schwannoma in the parapharyngeal space, and review the pertinent literature.
