We report the clinical course in a 25-year-old male patient of neurofibroma
tosis with malignant triton tumour (MTT). Triton tumour is a peripheral ner
ve sheath tumour with rhabdomyoblastic differentiation. This is relatively
rare tumour of head and neck region and only 26 cases have been reported to
date. The present case is the first reported MTT of parapharyngeal space,
one of the most aggressive course among all head and neck MTTs, resulting i
n the death of the patient within three months following surgical resection
. The aggressive nature of this tumour necessitates adjuvant therapeutic me
asures in addition to radical surgery in order to obtain better survival ra
tes.