Malignant schwannomas of the oral cavity and especially inferior denta
l nerve tumours, are rare malignancies. Mostly arising de novo as soli
tary tumours, some cases have occurred in Von Recklinghausen neurofibr
omatosis (25% of cases) or, as in the present report, at the time of a
relapse by transformation of a benign schwannoma. Long survival is on
ly observed in selected early diagnosed patients additionally treated
by extended surgery. Support of magnetic resonance imaging and immunoh
istological techniques in diagnosis are stressed. Advances in reconstr
uctive surgical devices after debulking surgery have contributed to en
hance the performance of more curative procedures (hemimandibulectomy,
intra oral resections) and to improve the functional and esthetic res
ults in these bad prognosis nerve tumours of the oral cavity.