Dyschromatosis universalis hereditaria (DUH) is a rare pigmentary genodermatosis mostly reported from Japan. It is usually characterized by widespread hyper/hypopigmented macules all over the body. Here, we report the case of a patient from Iran who presented with disseminated hyper and hypopigmented lesions over the trunk, neck, and extremities since the age of eight. To the best of our knowledge, to date, there has been only one reported case of DUH from Iran.