Coronary artery disease (CAD) is the leading cause of fatalities worldwide. Nuclear factor (NF)-kappa B is a transcription factor that controls cell proliferation, differentiation and immunity. To the best of our knowledge, the present study is the first investigation of the association between CAD and NF-kappa B1 -94 W/D/NF-kappa BIA 3'-untranslated region (3'-UTR) A -> G polymorphisms. The study population comprised 226 CAD patients and 201 controls. There was no significant difference in NF-kappa B1A 3'-UTR A -> G in the allele and genotype frequencies between case and control populations. The D allele frequency of NF-kappa B1 -94 in the case group was significantly higher compared to the control group (P= 0.028, odds ratio= 1.37). The genotype frequency of NF-kappa B1 -94 DD in the case group was significantly higher compared to the controls (P= 0.028). Linkage analysis showed a close linkage among these 2 genes (P< 0.001 for case and control), and AD and GD haplotypes were associated with CAD (P< 0.001; P= 0.015, respectively). NF-kappa B1 -94 DD genotype can be a significant risk factor for the development of CAD.