A case of enchondromatosis confirmed by the X-ray and microscpic findings, was reported, who was 25 year-oldman with the complaints of the globular mass deformities of both hands and the limping owing to the shortening ofright leg and the genu varum deformity of the lower extremities during about 21 years. His family history wasnon-contributory. The X-ray films of the chest, lumbar spine, and both extremities and hands, showed the varioussized cartilaginous globular mass es consisting with some of calcium deposits originating from the bony tissuessepecially in the metaphseal areas. We discussed that it should be differentiated from metaphyseal aclasia whichsimilar findings roentgenologically. He was operated for the removal of the masses the left 1st, 2nd 3rd fingers.After operation the micorscopic examination revealed that the specimen consisted of hyperplastic cartilaginoustissue with hyaline degeneration and large areas of myxomatous degeneration, but no evidence of any malignant changes.