Introduction and aim of work: Focal segmental glomerulosclerosis is the commonest underlying histopathological diagnosis in idiopathic steroid-resistant nephrotic syndrome in children. Many immunosuppressive therapies are used in its treatment. There is a 50% risk of progression to end-stage renal disease within five years if there is no complete or even partial remission with immunomodulatory therapies. This work aimed to study the various clinical, histopathological and therapeutic aspects of

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... idiopathic steroid-resistant nephrotic syndrome in our locality. Patients and Methods: This retrospective study was conducted on children aged 1 -15 years with idiopathic steroid-resistant nephrotic syndrome, followed in our Pediatric Nephrology Clinic in Sohag University Hospital, between January 2002 and January 2017. Patients' demographic features and disease's clinical course, histopathological patterns, response to various medications, and long-term outcomes were evaluated. Results: There were 28 patients with initial and 5 with late steroid resistance. The mean age at disease onset was 3.98±3.14 years. The male/female ratio was 2.5/1. Renal biopsy was performed in 26 patients. Minimal change disease was present in 5 patients, mesangioproliferative glomerulonephritis in 6 patients, and focal segmental glomerulosclerosis in 15 patients. Fifteen patients received cyclophosphamide, 26 received cyclosporine, 8 used mycophenolate mofetil, and 4 received combined immunosuppressive therapies. Four patients developed end-stage kidney disease. There were five deaths by the end of the study. Conclusion: In our study, focal segmental glomerulosclerosis is the most common histopathology in idiopathic steroid-resistant nephrotic syndrome and cyclosporine is the most effective second-line therapy in those patients