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Aim:Nephrotic syndrome (NS) is the most common childhood glomerular disease manifested by proteinuria, edema and hypoalbuminemia. The aim of this study was to examine children with primary NS in terms of their clinical laboratory and histopathological features, and to evaluate their treatment responses.Materials and Methods:Thirty-eight (21 boys/17 girls) patients followed up with primary NS were included in this study.Results:The mean age at diagnosis was 6.4 years. The histopathological diagnoses were focal segmental glomerulosclerosis (FSGS) in 17 patients, minimal change disease (MCD) in 8, membranoproliferative glomerulonephritis (MPGN) in 3, and membranous glomerulonephritis in 1 patient. Those patients with MPGN were older than those with MCD and FSGS (p=0.035). Twenty-four patients were steroid sensitive. Steroid response rates were 88% in those patients with MCD, 41% in patients with FSGS and 33% in those with MPGN. At their last visit, three patients (7.9%) were diagnosed with chronic kidney disease.Conclusion:NS is the most common glomerular disease of childhood. Early diagnosis and the histopathological features of this disease have an important place in its prognosis. Knowing the demographic, clinical and pathological features of the disease is helpful in monitoring its progress and its prognosis.