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Of renal biopsies from 300 children with idiopathic nephrotic syndrome (INS), 32 showed histological lesions of focal glomerular sclerosis. From this latter group, 25 had segmental and focal hyalinosis (FSH) with or without mesangial hypercellularity, and 7 focal glomerular obsolescence (FGO). The overall outcome after a mean observation of 7 years (0.5-18) was as follows: 11 patients (34%) were in complete remission, 12 (38%) had persistent proteinuria and/or recurrent nephrotic syndrome, 6 (19%) were in chronic renal failure and 3 patients (9%) died from non renal disease. Patients with FGO had a lower incidence of hematuria, hypertension and tubular defects, a better response to steroid and cyclophosphamide therapy and a better overall outcome. These results are similar to those reported by Habib and Gubler (Ped. Nephrology, Ed. Rubin and Barratt, p. 499-515) and indicate a more favorable outcome than that reported by Cameron et al. (Clin. Nephrol. 6:213, 1978). A progressive decrease in GFR was observed in some patients with persisting proteinuria or recurring nephrosis indicating that ultimate outcome of FGS in children cannot be established from pediatric age population studies.