Little is known about the natural history of scoliosis found in patients with syringomyelia, including the factors affecting scoliosis curve progression and the effect of syrinx drainage treatment. Twenty patients having scoliosis with syringomyelia diagnosed by MRI were followed up for 6.6 (range 2.0–12.6) years on an average. Various factors potentially influencing curve pattern or progression in these patients were then retrospectively reviewed. The convex side of major curve of scoliosis tended to be on the same side as the syrinx and as the unilateral neurologic abnormality. No correlation was found between the location and the size of the syrinx and the location and size of the major curve of the scoliosis, or between the severity of neurologic deficit and the size of the major curve of the scoliosis. In patients under the age of ten at the time of diagnosis of scoliosis and with a flexible curve, decompression of the syrinx improved or stabilized scoliosis. In most patients over the age of ten, surgical treatment of the scoliosis was necessary because of the large initial size of the curve or progression of the curve even after syrinx drainage. Other factors including gender, location of the syrinx, type of the curve, and severity of neurologic deficits did not correlate with the progression of the curve. The results of this retrospective study suggest that early diagnosis and decompression of a syrinx in scoliosis patients especially under the age of ten is crucial and may decrease the curve size and limit scoliosis curve progression.