Idiopathic herniation of the spinal cord is an extremely rare disorder which may cause progressive myelopathy. Two cases of this entity reported herein were both examined using MRI and CT myelography. The typical appearance of this disease with or without a dorsal intradural arachnoid cyst is focal ventral displacement of the mid-thoracic spinal cord, mimicking an isolated intradural spinal arachnoid cyst on MRI. CT myelography using thin slice sections is useful in the diagnosis of this disease.