A case of familial hemolytic uremic syndrome (HUS) in a four"year-old boy is reported. In his family four adult members are affected with the same disease. In the present patient we decided to perform bilateral nephrectomy because of the inexorable evolution of the disease. The intervention was followed by a distinct improvement, both from the clinical and the laboratory point of view. The child has profited of a renal transplantation. Unfortunately, he died three weeks later because of a severe urological complication. The aim of this study was to examine the effect of bilateral nephrectomy and transplantation on the clinical, laboratory and pathologic anatomical findings, and to analyze the literature, in order to find out whether these measures should be applied in the treatment of familial HUS with severe evolution. The observations seem to confirm the hypothesis of a renal pathogenesis of primary non endemic familial HUS. Nephrectomy seems to stop the disease, and the risk of a recurrence of the HUS after transplantation may be small. The occurrence of the disease in five family members in three generations was interpreted in favour of a genetic predisposition with a dominant gene.