Background and aim of work: Idiopathic nephrotic syndrome (INS) is a common and important chronic kidney disease in childhood. Steroid response pattern and the underlying histopathological spectrum of INS show variabilities in the different geographical regions. This work aimed to highlight the clinical profile, steroid response pattern and histopathological spectrum of INS in our locality in Sohag, Upper Egypt. Patients and Methods: A prospective study included 77 children with INS aged from 1-15 years followed for at least one year, diagnosed and treated at our pediatric nephrology unit in the period from January 2010 to December 2014 was done. The patients' demographic features, clinical profile, steroid response pattern and the underlying histopathological spectrum were studied. Results: Total included patients were 77 children. The mean age at disease onset was 4.87±2.94 (range 1.5-15) years. Male/female ratio was 1.6/1. Steroid was given to all patients, 79.2% of patients had steroid sensitive nephrotic syndrome (SSNS), while 20.8% had steroid resistant nephrotic syndrome (SRNS). Out of 61 steroid sensitive patients, about 54% developed either steroid dependent nephrotic syndrome (SDNS) or frequently relapsing nephrotic syndrome (FRNS). Biopsy was done in 33.8% of patients and revealed focal segmental glomerulosclerosis (FSGS) in 42%, minimal change disease (MCD) in 39% and mesangio-proliferative glomerulonephritis (MesPGN) in 19% of patients. End stage renal disease (ESRD) developed in 1 patient with SRNS and FSGS. Conclusion: About eighty percent of our patients were steroid sensitive but more than half of them had SDNS or FRNS. The main histopathological pattern seen in renal biopsies was FSGS.