AbstractMost nerve tumors (benign and malignant) do not arise from the nerves per se, but from the supporting cells; tumors arising from the cells of Schwann are termed schwannoma or neurilemmoma—benign or malignant. Surgical extirpation is the most effective treatment for these tumors. Radiation therapy can offer significant palliation and prolongation of life, but no cures have been observed. Benign tumors can be treated by local surgical extirpation; malignant tumors must be radically resected, including major amputation where indicated. Neurofibromatosis (von Reckling‐hausen's disease) is a genetic error of metabolism with a proclivity to produce multiple neurofibromas and, in about 10% of the patients, malignant neurilemmomas. Of 100 patients with malignant neurilemmomas treated by the author, 74 were considered determinate; among them, the 10‐year “cure” rate was 32%. Patients with von Recklinghausen's disease had almost as good a 10‐year survival rate as those with solitary malignant schwannoma (30% vs. 39%).