Hypophosphatemic vitamin D resistant rickets is a form of rickets characterized by typical structural deformities and roentgenographic and metabolic changes. it has a strong familial tendency and appear to be genetically transmitted. Authors experienced 7 patients with hypophosphatemic vitamin D resistant rickets which have been manifested through three generation in a family at Won-Kwng University Hospital from December 1982 to May 1984. Authors studied hypophosphatemic vitamin D resistant rickets clinically, radiologically biochemically and pathologically, and reported with review of literatures.