Erythrokeratodermas are inherited or sporadic disorders of keratinization, with many variants. Progressive symmetrical erythrokeratoderma presents as large, erythematous or hyperpigmented, hyperkeratotic, fixed symmetrical plaques over various parts of the body along with many other inconsistent features. Periorificial keratotic lesions have very rarely been reported in progressive symmetrical erythrokeratodermas. We report a 9 years old boy with progressive symmetrical erythrokeratoderma having perioral involvement besides fixed hyperpigmented, keratotic plaques. He was started with oral etretinate but he stopped the treatment due to no-response after two weeks and is presently using topical keratolytics without marked improvement.