There are a variety of tumors that can arise from various compartments and cellular components of peripheral nerves throughout the body. These peripheral nerve sheath tumors run the gamut from benign, fairly commonly encountered neoplasms such as schwannomas and neurofibromas to rarer, low grade neoplasms and variants such as perineuriomas, mucosal neuromas, palisaded encapsulated neuromas, granular cell tumors and nerve sheath myxomas to malignant neoplasms, so-called malignant peripheral nerve sheath tumors. They are generally classified as soft tissue neoplasms but they differ from most other tumors in this general grouping in a number of ways. Many of them are associated with genetic disorders or hereditary tumor syndromes and the majority of malignant peripheral nerve sheath tumors arise from a benign precursor tumor, neurofibroma. Their precise diagnosis and classification necessitates careful correlation with clinical and surgical data along with attention to histologic and immunohistochemical features. This text is comprised of a collection of chapters reviewing some of the myriad aspects of this group of neoplasms and includes discussions of the epithelioid variant of malignant peripheral nerve sheaths, peineuriomas arising in the colon, peripheral nerve sheath tumors arising in the oral cavity, jaw and salivary gland regions of the head and neck, and the melanotic variant of schwannoma.