Herniation of the spinal cord, or displacement of the cord outside the dura, is so rare that only 13 cases have been reported in the literature. The authors report a new case of spontaneous herniation of the spinal cord in a 38-year-old man who presented with lower left limb paresis and Brown-Séquard syndrome, with a T-8 sensory level. Displacement of the spinal cord was noted on computerized tomography following myelography and on magnetic resonance imaging. The herniated cord was confirmed at operation and reduced intradurally. Postoperatively, the patient showed complete neurological recovery. Based on a review of the literature, herniation of the spinal cord may be classified as spontaneous, iatrogenic, or traumatic. At cervical levels, the spinal cord has herniated into an iatrogenic pseudomeningocele located dorsally. At thoracic levels, spinal cord herniations were reported to be in a preexisting extradural arachnoid cyst located ventrally. The authors propose a pathogenesis for spinal cord herniation based on abnormal positioning of the spinal cord in the dural sleeve and the known anteroposterior movements of the cord that occur with cardiac and respiratory pulsations. The presence of a dural defect situated on the concavity of the spinal curvature is a prerequisite for this rare condition. As adhesions develop between the cord and the edges of the dural defect, cerebrospinal fluid pulsations push the cord into a preexisting cyst. The authors suggest modifying the classification by Nabors, et al., of spinal meningeal cysts to include this mechanism of spinal cord herniation. This diagnosis should also be considered in the differential diagnosis of myelopathy in the absence of a mass lesion.