Erythrokeratodermia variabilis (EKV) is a rare subtype of heterogeneous group of skin diseases called the erythrokeratodermi, and presents with migratory erythema and fixed hyperkeratotic plaques. EKV lesions commonly occur in the early stage of life. The lesions are hyperkeratotic and well-marginated and have a tendency to become confluent. EKV lesions are usually distributed on the extensor surface of extremities, buttocks, and face. Erythematosus lesions are transitory and show variation with stress and heat.Two patients aged 10 and 15 years were evaluated for erythematous and hyperkeratotic lesions that were clinically and histopathologically confirmed with EKV. We report these cases due to their rarity.The red spots initially spread centrifugally and disappeared without any mark, however, the brownish lesions on the anterior part of trunk and arms did not disappear. He was seen by different physicians and was treated with various fungal treatments that were not beneficial. No similar lesions were detected in other family members. No systemic disease was detected.