Rosette-forming glioneuronal tumors of the fourth ventricle are rare brain tumors, and only 19 such lesions have been previously reported. This report presents the first case of a rosette-forming glioneuronal tumors arising from the spinal cord. A 44-year-old woman presented with a 15-year history of dissociated sensory disturbance of the lower extremities that gradually spread through her upper extremities. She also experienced continuing motor disturbance. Magnetic resonance imaging demonstrated a mass in the cervicothoracic spinal cord that suggested an intramedullary spinal tumor. A total gross resection of the tumor was performed. As is typical of rosette-forming glioneuronal tumors of the fourth ventricle, this spinal cord example manifested neurocytic and astrocytic components. Neurocytic rosettes were detected in the neurocytic component, and the center of rosettes showed positive immunostaining for synaptophysin. The astrocytic component showed characteristic features of a pilocytic astrocytoma, as is often the case in the fourth ventricle examples.