Background and Aim: Vitiligo is a depigmenting skin disorder. Angiotensin converting enzyme (ACE) plays an important role in the physiology of the vasculature, blood pressure and inflammation. An insertion/deletion (I/D) polymorphism of a 287-base pair repetitive sequence in intron 16 of the ACE gene was reported to have been associated with the development of vitiligo. The aim of the present study is to study the possible association between ACE gene polymorphism and the development of vitiligo, to estimate the serum IL-6 and nitrite in vitiligo patients and to compare these factors in patients with different ACE genotypes. Subjects and Methods: This case-control study included 74 vitiligo patients and 75 apparentaly healthy controls. The distribution of ACE gene I/D genotype was investigated using PCR. Serum IL-6 and nitrite were measured by ELISA and Metal reduction methods respectively .Results: The ACE allele frequency was significantly different between vitiligo patients and healthy controls (P= 0.026). However there was no significant difference regarding the ACE genotype frequency between patients and controls (P= 0.115). There were a statistically significant higher VIDA score (P=0.007), IL-6 (P=0.000) and nitrite (P=0.007) in patients with the DD genotype when compared to other genotypes in vitiligo patients. Serum levels of IL-6 and nitrite in vitiligo patients were statistically significantly higher than those in controls.Conclusion: This study suggests that the ACE gene polymorphism confers susceptibility to vitiligo. An increase in the production of IL-6 and nitrite in vitiligo patients may play an important role in the aetiopathogenesis of the disease and may indicate a relation between these factors and different ACE genotypes