A case of highly malignant primary intracerebral schwannoma is presented in a boy aged 15 years. The histological, ultrastructural and immunocytochemical properties were consistent with a partly epithelioid schwannoma. All reports so far published of 18 intracerebral schwannomas were of benign tumors, one case was semi-malignant. As far as we know, this is the first report of a highly malignant intracerebral schwannoma.