Keratoderma of the palms and soles is not of rare occurrence. Its association with leukokeratosis of the mouth is well recognized. We describe two siblings having callosities like keratoderma over the weight bearing areas of soles, pressure areas of palms and palmar creases with leukokeratosis of cheeks. On study of the pedigree of these probands we could trace the trait transmission by a rare X-linked dominant mechanism.