Objective: It has been postulated that genetic predisposition may influence the susceptibility to infection and the disease outcome. The D allele of the angiotensin-converting enzyme (ACE) gene is associated with many diseases. However, there are only few reports available about infection. We have investigated the association between ACE I/D polymorphism and sepsis, its clinical features such as acute respiratory distress syndrome (ARDS), multiorgan dysfunction syndrome (MODS) and mortality. Material and Methods: Ninety-eight children who had been diagnosed with sepsis and 100 healthy individuals were included. The patients were divided into groups based on the presence of ARDS, MODS and survivor or nonsurvivor. The ACE gene polymorphism was analyzed by the polymerase chain reaction. Results: There was no statistical difference between the control and patient's genotype (p = 0.29). No evidence emerged regarding the association of ACE I/D polymorphism with MODS, but there was evidence of association with sepsis-related ARDS. It was found that carrying D/D genotypes increased the risk of the having ARDS 4.5 fold (95%CI 1.15-19.6, p = 0.028). On the other hand, there was not statistically significant difference between ACE gene polymorphism and mortality. Conclusion: In our study, the deletion polymorphism in angiotensin-converting enzyme gene is associated with increase in the risk of sepsis-related ARDS but not MODS and mortality in Turkish children.