Clinical and nephropathologic findings in autopsy material from 7 children with acquired immune deficiency syndrome (AIDS), 13 with severe combined immune deficiency (SCID), and 6 with a variety of other congenital immune deficiencies were reviewed in an effort to understand better the pathophysiology of the AIDS-related nephropathy. Non-HIV viral infection seemed to be associated with the development of the pathologic changes considered to be components of the AIDS-related nephropathy, and these changes, including focal segmental glomerulosclerosis (FSGS) and tubular epithelial cell injury and ectasia, were not limited to the kidneys of children with AIDS but were present in many of the congenital immune deficiencies. Of the 5 children with congenital AIDS, only the 3 who survived longer than a year developed AIDS-related nephropathy, whereas the two children with transfusion-acquired AIDS did not develop renal disease despite surviving for several years after initial infection.