Background : Renin-angiotensin-aldosterone system has been implicated in etiopathogenesis and progression of diabetic kidney disease (DKD). Strong evidence exists for the genetic predisposition in the development of DKD. The role of angiotensin converting enzyme (ACE) gene polymorphism in the susceptibility to nephropathy in type 1 diabetes (T1D) still debatable. Aim: We aimed to explore the potential association between ACE gene polymorphism and the risk of DKD in a cohort of Egyptian children with T1D. Research Design and Methods : Cross-sectional study included 102 children with T1D (54 males; aged 12-18 years) who were divided into normoalbuminuric-group (n=62) and albuminuric-group (n=40). ACE insertion/deletion (I/D) gene polymorphism was genotyped by polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) technique of the extracted genomic DNA from participants. Odds ratios (ORs) and 95% confidence intervals (CIs) were applied to explore the association between ACE gene polymorphism and the risk of DKD. Results: ‘D’ allele was significantly higher in albuminuric-group (risk-allele) (OR=7.4; CI=3.7-15; p<0.001), while ‘I’ allele was significantly higher in normoalbuminuicgroup (protective-allele). Binary logistic regression revealed that diabetic child with ‘D’ allele has 7.4-times higher odds to exhibit nephropathy compared to those with ‘I’ allele. D/D genotype was significantly higher in albuminuric-group (OR= 72.8; CI=11-482; p<0.001). Binary logistic regression revealed that diabetic children with D/D and I/D genotypes have 72.8-times and 4.7-times higher odds to exhibit nephropathy compared to those with I/I genotype, respectively. Conclusion: ACE D allele/DD genotype could be linked to increased risk for DKD, while ACE I allele/II genotype might be a protective factor for DKD among Egyptian children with T1D.