An unusual case of a giant intraspinal schwannoma in a 45-year-old woman with 14-year history of preoperative symptoms was presented. MRI of the spine revealed an intradural, extramedullary tumor extending from the intervertebral space C4/C5 to T4 vertebral body level (2 x 1.2 x 12 cm) and filling almost the entire spinal canal. Microscopical examination showed a typical neurinoma pattern with two distinct zones of Antoni A and Antoni B tissue. Some areas exhibited nuclear atypia and hyperchromasia reflecting the degenerative changes in this slowly growing nerve sheath tumor. A rich pericellular reticulin network was seen in the areas composed of Antoni A tissue. Immunohistochemically, the tumor cells were strongly positive for S-100 protein. The diagnostic difficulties in the presented case of longstanding schwannoma resulted in the late surgical treatment. The importance of the early diagnosis of spinal nerve sheath tumors for the patient's quick recovery is stressed.