Glomerular disease constitutes an important part of renal disease. The purpose of this study was to describe and correlate the types of glomerular diseases reported in native kidney biopsy specimens and their clinical presentations in Iranian children. The medical records and pathological diagnoses of patients with glomerular disease whose native kidney biopsy specimens were received by our hospital during a 10-year period were reviewed. In each patient, pathological diagnosis, clinical presentation, age at onset of disease, gender, and presence of nephrotic proteinuria, hematuria, and hypertension, were determined. Minimal-change disease was the most frequent glomerular disease and nephrotic syndrome was the most frequent clinical presentation encountered. For each disease, correlations between the clinical presentation and pathological diagnosis were made. This study describes various glomerular diseases encountered in Iranian children, with their respective clinical presentations.