A series of patients with abnormalities of the craniocervical junction with and without syringomyelia is reviewed. The impact of computerized tomography scanning on current radiological evaluation is discussed. Air myelography may no longer be necessary for accurate diagnosis. While the results of surgery for the Chiari malformation were good, at least one-half of the patients with syringomyelia showed continued progression of symptoms after posterior fossa surgery. More effective surgical therapy requires a better understanding of the pathogenesis of the syrinx.