Abstract Background: Systemic lupus erythematosus (SLE) is a complex autoimmune disease of unknown etiology. Objective: To assess serum levels of IL-17 in pediatric SLE (pSLE) and their association with clinical manifestations, laboratory findings, SLE disease activity index score (SLEDAI), and therapy. Patients and Methods: A case-control study was conducted at Pediatric Nephrology units, and Outpatient Nephrology Clinic of Children Hospital, Zagazig University. It was carried out over two years from September 2017 to August 2019. This study was performed on 60 patients classified into two groups: thirty of them were children with SLE and 30 apparently healthy children as a Control group of comparable age and gender. All patients were subjected to complete history taking and clinical examination. Laboratory investigations were done. Renal biopsy was done to 22 cases who were presented by renal symptoms. Results: Frequency of manifestation were malar rash 86.7%, photosensitivity 80.0%, renal manifestations, and anemia73.3% for each. SLEDAI score was 18 – 38. Class II lupus nephritis (LN) was the most common biopsies result (40%). The range of IL-17 for cases was (89.3 – 359 pg/ml), a highly significant increase in the Il-17 level in the case group than the control. A significant association between IL-17 level and renal manifestation, urea, creatinine, 24-hour urinary protein, and SLEDAI. Conclusions: Serum IL-17A is elevated more than 170 in pSLE patients with lupus nephritis, which correlates with disease activity and renal biopsy. IL-17 seems to have a possible role in the pathogenesis of lupus nephritis.