There is little information about the clinicopathological correlations of focal segmental glomerulosclerosis (FSGS) in children. Renal biopsies of 46 patients with primary FSGS were reviewed between 1994 and 2015. Clinical and laboratory findings were evaluated at the time of renal biopsy. Totally, not otherwise specified (NOS) was the most common pathologic variant in 38 patients. There was no correlation between age, gender, serum protein, urine protein, hematuria, and steroid response with five distinct pathologic variants and histopathologic lesions. Hgb level had a negative correlation to the glomerular hyalinosis, mesengial hypercellularity, mesengial deposition, and glomerular volume. Renal dysfunction and blood pressure had a significant positive association with the extent of tubular atrophy, interstitial fibrosis, synechiae in Bowman capsule, arteriolar hyalinosis, mononuclear infiltration, atherosclerosis, glomerular hyalinosis, mesengial hypercellularity, mesengial deposition, and glomerular sclerosis. In conclusion, serum creatinine and blood pressure were the significant predictors of histopathologic lesions in children with primary FSGS.