Intramedullary schwannomas account for 1.1% of all spinal schwannomas. Preoperative diagnosis is best accomplished by thoroughly evaluating clinical and radiological characteristics, accompanied by a high index of suspicion. The authors report a case of C2-3 intramedullary schwannoma in a young male who presented with neck pain and vertigo. The current literature is also reviewed. The authors reviewed the data of a young male with a 2-month history of neck pain and vertigo. Magnetic resonance imaging of the brain and cervical spine showed an intramedullary mass at C2-3 with a syrinx extending into the cervicomedullary junction. Laminectomy, myelotomy, and microsurgical excision of the mass under intraoperative neurological monitoring (IONM) were done. Postoperative pathology reported the specimen as a schwannoma. Gross-total resection of a schwannoma using IONM is the treatment of choice because of the lesion's benign nature, a better prognosis, and defined cleavage plane. Schwannomas should be included in the differential diagnosis of intramedullary spinal tumors. Because of its progressive nature, early surgery is recommended in symptomatic patients.