To determine the patterns in children with nephrotic syndrome (NS) in our region, we retrospectively studied 25 nephrotic patients evaluated and followed-up in the hospitals of the Aljouf region in Saudi Arabia. The male to female ratio was 2:1. The incidence of idiopathic NS was two to six cases per 100,000 children/year, while the prevalence was 12 cases per 100,000 children. Five patients presented with hypertension, seven (28%) with respiratory tract infection, three (12%) with tender abdomen, two (8%) with gross hematuria, one (4%) with thrombosis of renal veins with seizure and shock and the remaining seven presented to the hospital without complications. Twenty-three (92%) patients were sensitive to the first steroid course and two (8%) patients were steroid resistant, and both of them proved to have focal segmental glomerulosclerosis (FSGS) on biopsy. Of those who responded, six (24%) patients remained in remission, while 17 (68%) patients became steroid dependant. Of those who were diagnosed as steroid dependent, three patients were biopsied and one of them was diagnosed as FSGS, while the remaining two had minimal change glomerulonephritis. Regarding steroid-dependent patient relapses, seven (41%) patients showed infrequent relapses and ten (59%) patients had frequent relapses. We conclude that the patterns of NS and the response to treatment observed in this study did not differ significantly from studies from other places in the world.