Malignant peripheral nerve sheath tumours are very uncommon and can be found associated with neurofibramatosis Type I. Their prognosis is poor and surgery remains the treatment of choice. Radiotherapy and chemotherapy are second and third line of treatment. The diagnosis is based on clinical, radiological, histological and immunohistochemical tests. Malignant peripheral nerve sheath tumour have a very high potential of local recurrence and can even metastasize. They often occur in extremities but rarely can occur in brain. Very few cases have been reported in brain till date in the literature. We present here a case report of such a patient confirmed histologically and who had a residual disease post treatment.

Keywords: Intracerebral tumor; Rare case.