Syringomyelia was first recognized as a disease process some 400 years ago. The process of cystic dilation of the spinal cord is unpredictable and may result in a delay of many decades before the symptoms and signs of neurologic and orthopedic changes become apparent and commensurate with the observed cystic changes within the spinal cord. The syringomyelic process is usually associated with trauma, tumor, or congenital abnormalities at the craniocervical junction or along the spinal neuraxis. Several theories have been proposed as to processes involved in the development of spinal cyst formation, although none are completely compatible with the observed clinical pathology of syrinx development. Magnetic resonance imaging has markedly improved our ability to study the anatomy and natural history of syrinx formation, but to date, our understanding of the process remains imprecise. In view of the limited understanding of the pathophysiology of this disease process, it is not surprising that a variety of treatment regimens have been proposed and their efficacy remains difficult to fully evaluate.