Pseudoxanthoma. elasticum is a rare, heritable, systemic disease of connective tissue characterized by degeneration of elastic tissue and mainly affecting the skin, eyes and blood vessels. Recently, according to Pope (1974), it can be inherited as an autosomal dominant or recessive trait. We presented a study of 5 cases of pseudoxanthoma elasticum of autosomal recessive inheritance, which showed typical peau d'orange skin lesions. Two among the 5 cases were associated with angioid streaks, a case with myopia and broderline mental retardation, and 2 cases without any other systemic disturbances.