Progressive symmetric erythrokeratoderma (PSEK) is an autosomal dominant genodermatosis with incomplete penetrance and variable expressivity. It belongs to the group of erythrokeratodermas where it can be differentiated from erythrokeratoderma variabilis in the absence of migratory erythematous lesions and in a greater incidence of palmoplantar keratoderma. Molecular basis of PSEK has not yet been established although there are reports of mutations in the loricrin gene. We report a 13-year-old boy with symmetrically distributed hyperkeratotic plaques over the dorsum of the hands and the extensor aspect of the forearms, elbows and knees. As far as we are aware, we report the first case of PSEK in the Dominican Republic.