A patient with the nail patella syndrome (Fong's disease) with end stage renal failure received a renal transplant from his mother. There has been no recurrence of the disease in the year since transplantation. Such patients are apparently good candidates for transplantation, although careful selection of the donor from among the potentially affected family members should be conducted. In addition, the orthopedic and metabolic disabilities that characterize this disease may render these patients more susceptible to aseptic necrosis of the hips.