OBJECTIVES: Despite some studies suggesting a possible association between human leukocyte antigen (HLA), HLA-B*1502 and carbamazepine induced Stevens - Johnson syndrome (SJS) and Toxic Epidermal Necrolysis (TEN), the evidence of association and its magnitude remain inconclusive.  This study aims to systematically review and quantitatively synthesize the association between HLA-B*1502 allele and carbamazepine -induced SJS/TEN. METHODS: : A comprehensive search was performed in databases including Pubmed, Embase, CINAHL (Cumulative Index to Nursing and Allied Health Literature), IPA (International Pharmaceutical Abstract), HuGENet (Human Genome Epidemiology Network), Cochrane library and reference lists of studies, without language restriction. Databases were searched from their inception to July 2011. All studies were extracted by two independent authors. The primary analysis was the carrier frequency of HLA-B*1502 comparison between carbamazepine -induced SJS/TEN case and carbamazepine tolerant control. The summary odds ratios were calculated using a random-effects model. RESULTS: From 349 articles identified, 13 studies were included; 8 studies with 150 SJS/TEN cases and 498 matched-controls (carbamazepine tolerant control) and 5 studies with 45 SJS/TEN cases and 2,464 population-controls (general population). We found a statistically significant association between HLA-B*1502 allele in both groups of studies with matched-control (odds ratio (OR) = 124.18, 95% confidence interval (CI) = 47.48 - 324.77) and population-control (OR = 66.42, 95% CI = 9.20- 480.04). Subgroup analysis in matched-control studies according to ethnicity yielded similar and significant findings (Han-Chinese odds ratio (OR) = 236.24, 95% confidence interval (CI) = 71.72 - 778.11; Thai odds ratio (OR) = 54.43, 95% confidence interval (CI) = 16.28 – 181.96). CONCLUSIONS: We found a strong and significant association between HLA-B*1502 and carbamazepine -induced SJS/TEN. Therefore, HLA-B*1502 allele screening should be considered in population with high risk of SJS/TEN.