Peripheral nerve tumors are most often benign tumors of the nerve sheath; uncommonly they come from the nerve cells or are metastatic tumors. A precise diagnosis is required for well-adapted and effective treatment, as is good knowledge of fibromatosis diseases. In some cases, the diagnosis of the nerve tumor will lead to a diagnosis of phakomatosis. Surgical treatment must be clearly discussed, which, in case of schwannomas gives very good functional results. Primitive malignant tumors remain an unsolved therapeutic problem.