Dyschromatosis Universalis hereditaria is a rare pigmentary disorder presenting with dyschromia of skin involving trunk and extremities arranged in mottled manner lesions, usually present in the first few years of life. Herein, we report this extremely rare case of dyschromatosis universalis hereditaria in a middle-aged male with a family history of dyschromia in his younger sister, now presenting to us with a keratoacanthoma.