A 9-year-old boy presented with the numbness in both arms and hands, and neck stiffness. On examination, he had a slight quadriparesis and restricted neck movements. There were no signs of von Recklinghausen's disease. Magnetic resonance imaging (MRI) scan disclosed a gadolinium enhanced intramedullary tumor located at C6-T1 associated with syringomyelia. C6-T1 laminectomies were performed and the intramedullary tumor was totally removed by a microsurgical technique. Postoperative course was uneventful. The pathological examination revealed an intramedullary schwannoma. The occurrence of intramedullary schwannoma in a patient without signs of von Recklinghausen's disease is extremely rare. We have been able to find 57 cases of intramedullary schwannoma reported in the literature. Intramedullary schwannomas are usually seen in males. The ages of the patients ranged from 9 to 75 years (mean 40.44 years). Only 4 cases in the pediatric age group have been reported. The duration of symptoms ranged from 3 months to 20 years (mean 31.03 months). Symptoms and signs varied with the location of tumor. The vertebral levels of intramedullary schwannomas were usually cervical (61%). MRI has been the choice of diagnostic tool in the cases reported since 1986. The majority of the cases showed either a partial or complete recovery in the postoperative period.