We report a Japanese patient with skin-coloured keratotic papules on the hands and feet, and pronounced hyperkeratosis of the palms and soles. There was no evidence of fragmentation of dermal elastic tissue on histology. Freckle-like pigmentation was also present on the dorsa of the hands and feet, and this appeared to be closely related to the keratotic disorder. The mode of inheritance was autosomal dominant. We consider our case to be a new variant of familial punctate acrokeratoderma, associated with a pigmentary disorder.