Recent data suggest that the histologic finding of focal and segmental glomerulosclerosis (FSGS) is increasing among children. There are, however, limited longitudinal pediatric data on prevalence, demographics, and steroid responsiveness in FSGS. We identified 201 consecutive nephrotic children diagnosed between 1977 and 2002 with 2 years follow-up; 51% had undergone renal biopsy due to steroid sequelae or resistance; 48 children with FSGS were diagnosed. Compared with non-FSGS children, FSGS children were older at diagnosis (6.9 years vs 4.4 years, P<0.02), more likely girls (54% vs 28%, P<0.02), Black or Hispanic (42% vs 16%, P<0.001), and the FSGS was more likely to be steroid resistant (73% vs 10%, P<0.001). To assess for longitudinal differences, we grouped children by presentation: pre-1985, between 1985 and 1995, and post-1995. There was no difference in proportion of children biopsied or diagnosed with FSGS during each interval. Among FSGS children, there was no difference in racial or gender composition in each period, but there was a difference in age at diagnosis (2.6 vs 5.7 vs 8.5 years; P=0.01), also observed in the non-FSGS children (2.2 vs 3.9 vs 4.9 years; P=0.02). In contradistinction to non-FSGS children, there was a marked increase in steroid resistance with FSGS (43% vs 62% vs 86%; P=0.03).