Background : Lupus nephritis is a major contributor to morbidity and mortality in patients with systemic lupus erythematosus (SLE). Biopsy proven lupus nephritis (L.N.) occur in a round 80% of all cases of childhood onset SLE. The etiology of SLE remains unknown. However, there is evidence that both genetic and environmental factors play a role in disease development. Monocyte chemoattractant protein-1 (MCP-1) is a chemokine (CC) that is mainly expressed by activated monocytes / macrophages leucocytes and other mediators into sites of inflammation. There is evidence that polymorphisms in the genes of inflammatory mediators may predispose to the development of L.N. Aim of the work : Our study aimed to examine role of a functional MCP-1 polymorphism in children with L.N. Subject and Method : this study was conducted on 50 child, they included 40 SLE patients ( ≥4 American collage of Rheumatology (ACR) criteria for SLE) 20 with and 20 without L.N.) and 10 normal controls age and sex matched. DNA was obtained and MCP-1 genomic variants were detected by polymerase chain reaction (PCR) followed by Restriction Enzyme Fragment Length Polymorphism (REFLP). Results : The A/A genotype was more common in control (80%) than SLE patients (52.5%), where as both A/G & G/G genotypes were more frequent in SLE patients (17.5% & 30%) than in control (0% & 20%) (P = 0.215 for all). The A/A genotype was observed in only 45% of the patients with LN compared with 60% of those without LN (P>0.05). The frequency of A allele in patients without L.N. (65%) was more common than in patients with L.N. (57.5%) while frequency of G allele in patients with L.N. (42.5%) was more common than in patients without L.N. (35%). (P>0.05). Conclusion : These results may suggest a possible role of genetic polymorphism of MCP-1 in the development SLE and subsequently L.N. in children which still need further investigation.