The Receptor for Advanced Glycation End products (RAGE) is a multiligand receptor with a potential role in the atherogenic process in diabetes. The polymorphisms -429 T>C, -374 T>A and a 63 bp deletion (63 Del) of the promoter region of the RAGE gene were previously described and showed to increase gene transcription. The aim of this study was to determine the frequency of these polymorphisms in a sample of Brazilian patients with type 1 Diabetes mellitus (T1DM). The University Committee for Ethics in Human Research approved this study. RESEARCH DESIGN AND METHODS – The sample consisted of 104 patients (39.4% men) with T1DM; 72% Euro-Brazilians and 28% Afro-Brazilians. The mean age was 24.3 ± 7.1 years and blood levels of fasting glucose and glycated hemoglobin (A1C) were 219.5 ± 114.9 mg/dL and 9.9 ± 2.3%, respectively. Genotyping was performed by PCR-RFLP using AluI (for -429 T>C) and Tsp509I (for -374 T>A). The restriction fragments were separated by polyacrylamide gel electrophoresis and were stained with ethidium bromide. RESULTS - No significant difference (P > 0.18) in the genotype frequencies was observed between Euro- (N = 74) and Afro-Brazilians (N = 30) with T1DM. The genotype and allele frequencies of the total sample for -429 T>C were (65.3% TT; 32.7% TC; 2.0% CC; C = 0.184); and for -374 T>A were (44.1% TT; 47.1% TA; 8.8% AA; A = 0.33). All genotype frequencies met Hardy-Weinberg equilibrium expectations (P > 0.33). Two Euro-Brazilians (1.9%) had the 63 Del in heterozygous form. CONCLUSION – The genotype frequencies of the polymorphisms -429 T>C and -374 T>A observed in this study were similar to that of Finnish T1DM and Brazilian nondiabetic samples reported previously. This is the first report on these polymorphisms in T1DM Brazilian subjects.