Erythrokeratodermia variabilis is a rare genodermatosis characterized by the coexistence of randomly occurring, transient, erythematous patches and hyperkeratosis of the skin. A 25-year-old Uzbekistanian female with congenital deaf-mutism presented with well demarcated geographic hyperkeratotic patches and bizarre erythema on the whole body surface except the face, hands and feet from birth. The histopathological examination revealed marked hyperkeratosis, moderate acanthosis in the epidermis and vascular dilatation with a perivascular infiltration of inflammatory cells in the upper dermis. Herein we report on a rare case of erythrokeratodermia variabilis with congenital deaf-mutism which was rapidly improved by administering acitretin. (Korean J Dermatol 2011;49(4):379∼381)