A cohort of 263 children with perinatally transmitted HIV infection was followed prospectively at the University of Miami/Jackson Children's Hospital for the development of overt HIV infection and associated nephropathy (HIVN). Definitive HIV infection was confirmed by persistent titers of HIV antibodies and/or positive viral PCR assays after 18 months. Nephropathy was defined in patients determined to have persistent proteinuria greater than or equal to 1+ on urinary Dipstick(R) in the absence of fever on consecutive urines at least 2 weeks apart. Quantitation of proteinuria was performed by timed collections(UprV) or urine protein: creatinine ratios (Upr/Ucr). Significant proteinuria was defined as a UprV≥0.1 gram/m2/day or Upr/Ucr≥0.2. Nephrotic proteinuria was defined as UprV≥1 gram/m2/day or Upr/Ucr≥2. The racial and ethnic demographics of the population were predominantly Black(74%) with the remainder Hispanic (14%) and Caucasian (12%). 72 of the 263 children developed active HIV infection. Of these, 44 had persistent proteinuria making the actual incidence of HIVN as high as 54%. Twelve of the 44 (16.7%) had nephrotic range proteinuria. The overall mortality for the HIV+ children not attributable to renal disease has been 50% over 5 years. Only 6(13.6%) have progressed to end stage renal disease (ESRD) requiring dialysis. All were of African-American or Haitian descent. The renal pathology in all ESRD patients was focal glomerulosclerosis with nephrotic range proteinuria preceding the deterioration to ESRD. The mortality of the HIV+ dialysis patients was not different from the rest of the HIV+ children in the study. These data suggest a strong influence of race and ethnicity in the development and progression of HIVN in the pediatric population.