• Incontinentia pigmenti is a rare condition, which may involve the central nervous system. Computed tomographic (CT) examination can supply important information about the dynamics and pathologic nature of this poorly understood syndrome. Two patients suffering from incontinentia pigmenti were examined by CT because of central nervous system symptoms. Large low-density areas in the brain were demonstrated in the CT examination of the first patient a few days after birth, suggesting a local edema. Diffuse brain atrophy was found two years later. Brain atrophy was also found in the second patient in a CT examination done at the age of 11 years.