Intraspinal nerve sheath tumors, also known as intradural Chewang cell tumors, account for approximately 25% of intradural spinal tumors and can occur in all segments of the spinal canal, mostly solitary, with a peak incidence between the ages of 40 and 60 years, with no significant differences between the sexes. Intraspinal nerve sheath tumors originate from the dorsal spinal nerve roots and may produce submural infiltration when they grow centripetally, which is more common in cases of rhombic neurofibroma. Nerve sheath tumors of the brachial or lumbar plexus can grow along multiple nerve roots into the dura mater. In contrast, paravertebral Schwann cell tumors that extend into the spinal canal are usually located outside the dura. Approximately 2.5% of intradural nerve sheath tumors are malignant, and at least half of these occur in patients with multiple neurofibromatosis. The exact cause of tumor formation remains unclear, and there is considerable agreement that tumor development and growth are primarily due to molecular alterations at the genetic level. The vast majority of intradural nerve sheath tumors are located entirely within the dura, while 10%-15% grow outside the spinal canal through the dorsal nerve root cuff to form a dumbbell pattern; approximately 10% of nerve sheath tumors are located outside the dura or paravertebral; approximately 1% of nerve sheath tumors grow within the medulla and are considered to be growing along the nerve sheath around the vessels entering the spinal cord. Diagnosis The presence of significant neurogenic pain, motor and sensory deficits developing from the bottom up, an area of cutaneous hypersensitivity at the level of the tumor segment, and especially the presence of spinal cord hemisection syndrome (manifested as ipsilateral upper motor neuron motor palsy and hyperalgesia of touch and deep sensation below the lesioned segment, and contralateral loss of pain and warmth) as well as altered cerebrospinal fluid dynamics causing increased pain suggest the possibility of extramedullary spinal cord nerve sheath tumor. The diagnosis of extramedullary nerve sheath tumor should be confirmed by the necessary ancillary tests. The treatment of benign nerve sheath tumors is mainly surgical resection. The majority of cases can be cured by standard posterior laminectomy and total resection of the tumor, and recurrence is usually rare. Adjuvant radiation therapy is recommended after surgical resection of malignant nerve sheath tumors. Prognosis The prognosis for malignant nerve sheath tumors is extremely poor, with survival rarely exceeding one year. These tumors must be distinguished from some of the few Chewang cell tumors that exhibit aggressive histologic features, and those with a malignant tendency have a relatively good prognosis.