Several renal and renal-related disturbances have been described in patients with AIDS (acquired immune deficiency syndrome), in adults and children as well. These are mainly electrolyte and acid-base imbalance, acute renal failure and nephrotic syndrome. The latter is usually steroid non-responder. The renal histopathological lesions described more commonly are minimal change, mesangial hyperplasia and focal segmental glomerulosclerosis. Herein, we describe a 5 year-old with AIDS, that developed nephrotic syndrome, characterized by edema, ascites, hypoalbuminemia and massive proteinuria. A percutaneous renal biopsy showed mesangial proliferation. She did not respond to a 6 week treatment with prednisone. She died with sepsis after several viral and bacterial infections.