We present the case of a primary solitary malignant schwannoma of the trigeminal nerve. A total of 55 cases have been described in the literature; however, in these cases two tumors were affecting the supraorbital branch. This nerve-sheath tumor usually affects men in the fifth decade of life. The main clinical sign of malignant schwannomas of the head and neck is an indolent swelling. Hematogenic or lymphogenic metastasis has not been described. Because of the pleomorphism of the tumor cells immunohistochemical study is important. The treatment of choice is radical resection, possibly with adjuvant radio- or chemotherapy. The 5-year survival rate of malignant schwannoma of the trigeminal nerve is 41.7%.
