Some studies have reported that the HLA-DRB1 allele was associated with idiopathic dilated cardiomyopathy. However, there have been inconsistent results among different studies. To clarify the association of HLA-DRB1 and idiopathic dilated cardiomyopathy, a meta-analysis of case-control studies was performed.PubMed database, Science Citation Index database, The Cochrane Central Register of Controlled Trials database, China National Knowledge Information database, Chinese Biomedical Literature database, Wanfang database, and VIP database in China were searched. Search terms included dilated cardiomyopathy and DRB1. Five case-control studies were included in the present meta-analysis to assess the association between HLA-DRB1*1401, HLA-DRB1*0901, HLA-DRB1*0301, and idiopathic dilated cardiomyopathy.A total of four studies were included in our meta-analysis for HLA-DRB1*1401 and HLA-DRB1*0901. The pooled odds ratio (OR) was 2.6 [95% confidence interval (CI) 1.11-6.11, P<0.05] and 0.70 (95% CI 0.48-1.00, P=0.05), respectively. For the HLA-DRB1*0301 allele, just three studies were included in our meta-analysis. The pooled OR was 0.49 (95% CI 0.27-0.91, P<0.05). The present meta-analysis indicated that the frequency of HLA-DRB1*1401 was higher in idiopathic dilated cardiomyopathy patients than in healthy people, whereas HLA-DRB1*0901 and HLA-DRB1*0301 were higher in healthy people than in idiopathic dilated cardiomyopathy patients.The HLA-DRB1*1401 allele might be a risk factor for idiopathic dilated cardiomyopathy and HLA-DRB1*0901 and HLA-DRB1*0301 might protect humans from idiopathic dilated cardiomyopathy.