Abstract
Our case is an 8 years old boy in whom the skin change was found a week after birth. Initially the involvement was limited to limbs but gradually it involved whole body skin. The skin manifestations are composed of hyper and hypo pigmented patch whitin each other. Pathologic finding was compatible with dyschromatosis universals hereditharia. This disease is a rare pigmentary disorder which is characterized with irregular hyper and hypo pigmented macula and patch in a reticular pattern. Skin manifestation can be limited to limbs or can be diffuse in all body skin. The lesions begin mostly several weeks after birth until 6 year of age.