Hypophosphatemic vitamin D resistant rickets is a form of rickets characterized by typical structuraldeformities and roentgenographic and metabolic changes. It has a strong familial tendency and appear to begenetically transmitted. Authors experienced 7 patients with hypophosphatemic vitamin D resistant rickets whichhave been manifested through three generation in a family at Won-Kwang University Hospital from Dec.1982 to May1984. Authors studied hypophosphatemic vitamin D resistant rickets clinically, radiologically, biochemically andpathologically, and reported with review of literatures.