Dyschromatosissymmetricahereditaria (DSH) is one of a group of reticulate pigment disorders of the skin. It is a rare autosomal dominantly inherited genodermatosis, presenting as mottled admixtures of hypopigmented and hyperpigmented macules on the dorsa of the extremities. It is predominantly found among persons of Oriental origin. We hereby document the first case of dyschromatosissymmetricahereditaria in a four year old Nigerian boy who presented with progressive symmetric dyschromic lesions on the dorsa of the hands and feet, with no family history of similar lesions. The diagnosis was confirmed by the typical histologic finding of basal hyperpigmentation with normal number of melanocytes and absence of melanin incontinence from a hyperpigmented lesion