Cockaynes syndrome is rare familial, autosomal recessive disorder. Clinical characteristics are growth and mental retardation. There starts at birth, usually 2nd year of life. Radiologidal findings are as follows; microcephaly, dense calvarium, and abnormal intracranial calcification with hydrocephalus. Skeletal abnormality including slender ribs, decreased height of vertebral bodies and contour abnormality such as narrow medullary canal in long bone with osteoporosis, small squared pelvis, abnormal sclerotic foci and disequibrium in diaphysio-epiphysis in foot. The authors report a case of cockaynes syndrome with clinical and roentgenologic findings.