Nerve tumors present as palpable swelling (lump), sensory and/or motor deficits, tenderness to palpation, or neuropathic pain. In 91% of cases, tumors are benign nerve sheath tumors like neurofibromas and schwannomas. Multiple neurofibromas or schwannomas occur in neurogenetic syndromes like neurofibromatosis or schwannomatosis. Perineuriomas, lipomas, aggressive fibrosis (desmoid tumors), paragangliomas, and hemangiomas are even more rare nerve tumors that do not necessarily originate from the nerve sheath. The correct classification of potentially malignant lesions such as MPNST (malignant peripheral nerve sheath tumor) or intermediate stages such as ANNUBPs (atypical neurofibromatous neoplasms with unknown biological potential) that may occur in neurofibromatosis type I may be challenging. Misdiagnosis and mistreatment can lead to severe deficits and pain syndromes. Imaging, intraoperative neuromonitoring, clinical and surgical experience, as well as an interdisciplinarity approach may help to achieve good treatment results.