Background: Angiotensin II (Ang II) is found to perpetuate inflammation and visceral damage in systemic lupus erythematosus (SLE). It mediates most of its actions through Ang II receptor type I (AT1) whose gene polymorphism A1166C (CC genotype) seems to have pathogenic effects. Objective: To measure serum Ang II and the frequency of AT1 receptor CC genotype among a group of Egyptian patients with pediatric onset lupus nephritis (pLN). Methods: This is a case-control cross sectional study which included 24 patients with pLN and 24 age and sex-matched healthy subjects as controls. Clinical evaluation and routine laboratory markers for SLE patients were done. SLE disease activity index (SLEDAI) and the British Isles Lupus Assessment Group (BILAG)-2004 renal score were measured. Serum Ang II was measured by enzyme linked immunosorbent assay and detection of ATI receptor CC genotype by polymerase chain reaction were done for both patients and controls. Results: Patients had significantly higher serum Ang II than the controls (p=0.0001). The frequency of AT1 receptor CC genotype was significantly higher among patients as compared to the control group (p=0.008). Both serum Ang II and AT1 receptor CC genotype were comparable between patients with proliferative LN class III and IV and those with LN class II (p>0.05). Serum Ang II did not correlate significantly with SLEDAI or BILAG-renal score (p>0.05). Conclusion: Serum Ang II and AT1 receptor CC genotype seem to have pathogenic role in pLN but with no deleterious effects on the phenotype of LN for further assessment.