Management of intramedullary spinal cord lesions still represents a formidable challenge to neurosurgeons. The scope of intramedullary lesions of the spinal cord demanding surgical and/or medical intervention is wide. With this in mind, we have attempted to study the various pathologies affecting the spinal cord, seen commonly in neurosurgical practice, and attempted to evaluate the best available diagnostic and therapeutic techniques to provide the best outcome for the patients.