Idiopathic (primary) tubulointerstitial nephritis (TIN) of childhood is relatively rare. Four children, two with concomitant uveitis, aged 8-14 years, with idiopathic TIN who underwent repeat renal biopsy were retrospectively evaluated. At presentation, all had a significant elevation of the urinary beta(2)-microglobulin/creatinine ratio (beta2MG ratio), ranging from 10100 to 44550, with increased histological indices of tubulointerstitial scores (TI scores) in excess of 6 points. Three of the children received prednisolone (PSL) therapy following diagnosis, while the remaining child received the therapy 30 months after the first renal biopsy. In the children that received prompt PSL therapy, a rapid decrease in urinary beta2MG ratio was observed and the TI scores obtained at a mean interval of 16 months after the first biopsy decreased to less than 5, while preserving renal function. In the remaining child that received delayed PSL therapy, persistent elevations of urinary beta2MG ratio and TI scores were observed. He subsequently progressed to chronic renal insufficiency. These clinical findings suggest that persistent elevations of urinary beta2MG ratio and TI scores are indicators of progression of renal failure in TIN. For successful treatment, early therapeutic intervention should be deployed in selected patients with severe idiopathic TIN.