The neuropsychological abilities of children with congenital ( n=13) or acquired ( n=11) end-stage renal disease (ESRD) were compared. Patients were being treated with or being prepared for dialysis and were awaiting transplantation. None of the children had an identifiable syndrome with associated central nervous system (CNS) dysfunction or had exposure to drugs with known CNS toxicity. There were no group differences in intelligence, academic achievement, behavior, or immediate memory. Children with congenital ESRD had poorer fine motor coordination and more difficulty on tests of verbal and nonverbal long-term memory than children with acquired ESRD. However, the neuropsychological outcome for congenital ESRD is more favorable than previously described. Psychological and education treatment recommendations should be considered.