Osteopoikilosis is a rare disease of the bone that is occasionally associated with skin lesions. The latter have been reported as dermatofibrosis lenticularis disseminata, juvenile elastoma, and connective-tissue nevus. The occurrence of this syndrome in three members of a family is used to demonstrate the salient features of familial incidence, characteristic distribution patterns, and histologic variations. The clinical similarities between variously reported cases and recent work at the ultrastructural level offer an explanation of the histologic variations found in these lesions. There is confusion associated with the nomenclature and a classification is offered that permits the identification of dermatofibrosis lenticularis disseminata as a type of connective-tissue nevus.