The aim of this publication is to present a case of idiopathic spinal cord herniation (ISCH) associated with a transdural disk herniation, demonstrate an operative technique used to treat this condition and provide an updated review the literature. ISCH is an infrequent condition that can cause progressive myelopathy leading to severe neurological dysfunction. This condition is characterized by ventral displacement of the spinal cord across a defect in the dura, either congenital or acquired, resulting in vascular compromise and adhesion that subsequently causes injury to the spinal cord. We present the management of such a patient, in addition to a review of the literature regarding management of ISCH. This patient underwent surgery using the dural graft sling technique for repair of the dural defect and restoration of normal spinal cord position within the thecal sac. A review of the literature revealed a total of 171 patients supplemented by our 1 patient, which were then analyzed. The majority of patients, treated with a variety of surgical techniques, experienced improvements in symptomatology. Our patient experienced significant improvement in symptomatology. Although ISCH is a rare clinical condition that causes myelopathy, patients managed with surgery generally, though not universally, have a favorable neurological outcome. The associated surgical technique video demonstrates the dural sling technique for the treatment of this rare disorder.