Abstract Three cases of Cockayne's syndrome are analyzed to describe the characteristic radiographic findings. There is a progression of microcephaly, with thickening of the cranial vault and intracranial calcification. Diaphyseal constriction of the long bones, flattening of the vertebral bodies, and disproportionately large extremities are common findings. The syndrome presents as a growth retardation within the first two years of life. Subsequently, there is regression toward infantile behavior and mental retardation. Although the etiology is unknown, the radiographic findings are pathognomonic for this abnormality.