In this report, a case is presented of a child, descendent from parents originating in the Virgin Islands, with symmetric and progressive hyperpigmented, hyperkeratotic plaques consistent with progressive symmetric erythrokeratoderma (PSEK). Additional family members were also affected in an autosomal dominant pattern of inheritance. Erythrokeratodermas are rare genodermatoses that have characteristic clinical presentations of well-demarcated, hyperkeratotic, and erythematous plaques. Three types exist, differentiated by their clinical presentation. In this report, a case of progressive symmetric erythrokeratoderma (PSEK) is presented. The clinical features, pathogenesis, and treatment options for erythrokeratodermas are discussed.