Sixty patients with diastematomyelia were seen over a thirty-year period and congenital scoliosis was found in 60 per cent. All of the patients had associated vertebral abnormalities and most (87 per cent) had a neural deficit. Myelography was helpful in the diagnosis, particularly prior to any procedure that might cause traction on the spinal cord. Laminectomy for removal of the spur was indicated when neural deficits were progressive or before corrective surgery on the spine, and in ten patients the operation alleviated neural sequelae. Observation of patients with diastematomyelia who have no neural deficit or a stable, non-progressing deficit is recommended.