Dyschromatosis universalis hereditaria (DUH) is a rare genodermatosis mainly described in asian subjects. Here, we report a case of a caucasian 11-year-old boy with DUH and an unaffected twin brother. Parents were not consanguineous. A review of the main phenotical, clinical and hystological aspects of this rare entity is exhibited. Differential diagnose might be stablished with several pigmentary disorders, so Dermatologist might have this entity in mind to make a correct diagnose, specially in cases with no response to typical treatments.