〔Abstract〕 Objective To investigate the association between endothelin receptor type A gene (EDNRA) and congenital bilateral absence of the vas deferens (CBAVD). Methods This case-control study consisted of 124 subjects with CBAVD and 100 fertile controls. Results Two single nucleotide polymorphisms (SNPs: rs5335 and rs1801708) in the EDNRA gene were genotyped via PCR, PCR-RFLP analysis, and direct sequencing. No significant differences existed between EDNRA polymorphisms and CBAVD phenotype (rs1801708: P=0.220 2, 0.163 2; rs5335: P=0.805 8, 0.818 6). However, the rs1801708-rs5335 haplotype AG was notably associated with an increased risk of CBAVD (P=0.0086, OR=2.178, 95% CI: 1.207-3.929). A significantly protective effect of rs1801708-rs5335 haplotype GG on CBAVD (P=0.0385, OR= 0.671, 95% CI: 0.460-0.980) was observed. After Bonferroni correction, the result for haplotype A-G remained significant (P =0.008 558 < 0.0125), while haplotype G-G was not significant. Conclusion The rs1801708-rs5335 AG haplotype of EDNRA is a potential risk factor for CBAVD development in Han Chinese.