Introduction and Aim: The studies investigating epidemiologic, clinical and histopathological findings related to childhood renal disease are scarce in our country. This study aims to examine the clinical and histopathological profile of the renal biopsies evaluated at the Pathology Department of Behcet Uz Children's hospital (BUCH) and determine their correlation with clinical diagnosis. Material and Methods: A Retrospective descriptive study. The documents of the 123 biopsies performed between 2009-2010 at Izmir Dr. Behcet Uz Children's Hospital and Izmir Tepecik Training and Research Hospital Child Health Department, and evaluated at the BUCH pathology department were assessed. All patients were analysed based on their age, sex, systemic disease, familial history of renal disease, indications of biopsy, clinical diagnosis, hematuria, proteinuria and some relevant laboratory findings. 110 biopsies, which were considered as adequate for pathological evaluation were subjwected to immune fluorescent (IF), and light microscopic examination. Results: The mean age was 9.4 +/- 4.0 years, and 52% of the patients were females. Among 170 biopsy indications the leading one was nephrotic proteinuria with a frequency of 51.8 percent. Among 201 clinical diagnoses, FSGS ranked first with 25.9%. Mezengioproliferative glomerulonephritis, minimal lesion disease which was reported as normal under light microscopic evaluation, and nondiagnosed specimens which were only reported with their microscopic features were the three leading conditions of the histopathological evaluations. Clinicopathological correlation was achieved among 86.3% of the patients while this correlation was lost for cases with tubulointersititial nephritis and chronic glomerulonephritis. Conclusion: In this study renal biopsy indications were consistent with the literature and nephrotic syndrome, proteinuria and hematuria were the leading indications. Among the nephrotic syndrome group mesengioproliferative glomerulonefritis and ML disease are predominant, while in the hematuria group the leading pathological diagnoses were HSV nephritis and IgA nephropathy which were in accordance with the literature. For achieving better clinicopathological correlation, electrone microscope evaluation, and genetic analysis are required.