A total of 376 renal biopsies performed in several hospitals in Riyadh, on children with glomerulonephritis were reviewed. Focal segmental glomerulosclerosis (FSGS) was the most common glomerulopathy found and accounted for 120 cases (31.9%), followed by mesangial proliferative glomerulonephritis in 99 (26.3%). Minimal change nephrotic syndrome was seen in 55 (14.6%), membranoproliferative glomerulonephritis in 30 (8.0%), membranous glomerulonephritis in 18 (4.8%), IgA nephropathy in 15 (4.0%), post-infectious glomerulonephritis in 15 (4.0%), Alport syndrome in 14 (3.7%) and rapidly progressive glomerulonephritis in 10 (2.7%). Our findings show a high prevalence of FSGS in comparison with most international renal pathological studies published in children. At the same time, there is a relatively low prevalence of IgA nephropathy as compared to some other Asian countries. Additional, more comprehensive, clinical and pathological studies on Saudi children with glomerular diseases are needed to confirm or negate these findings. Environmental and genetic factors have to be explored and studied for their role related to these differences. Also, there is an urgent need to establish a glomerulonephritis registry for children in Saudi Arabia.