We report the clinical course in a 25-year-old male patient of neurofibromatosis with malignant triton tumour (MTT). Triton tumour is a peripheral nerve 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 in the death of the patient within three months following surgical resection. The aggressive nature of this tumour necessitates adjuvant therapeutic measures in addition to radical surgery in order to obtain better survival rates.