Abstract: 　Objective　To explore the correlation between the polymorphism of exon 10 (ABCA3 exon10) in gene of ABCA3 and the incidence of respiratory distress syndrome (NRDS) in the Han nationality in Western Inner Mongolia. Method　Fiftythree Han children with NRDS hospitalized from September 2014 to September 2016 were selected as case group. At the same time, 53 Han children without NRDS in the same area were served as control group. The DNA in both groups was extracted and polymerase chain reaction (PCR) was used to amplify ABCA3 exon10, and the amplified products were directly sequenced and the results were analyzed. Results　At the rs13332514 (F353F) site of exon 10 of ABCA3, there was a single base point mutation C>T, the codon was changed from TTC to TTT, and the amino acid at the 353rd coding site did not change. There was a point mutation in both the case group and the control group, and the variation rate of the case group was 30.2%, which was higher than that of the control group (13.2%), and there was significant difference (P<0.05). In NRDS group, 3 genotypes (CC, CT and TT) were detected at ABCA3 exon10 rs13332514 (F353F) loci, the frequencies were 69.8%, 20.8% and 9.4%, respectively. The allele frequency of C was 80.2%, and the frequency of T allele was 19.8%. In the control group, 2 genotypes (CC and CT) were detected, the frequency was 86.8% and 13.2%, and the allele frequency of C was 93.4%, and the frequency of T allele was 6.6%. The frequency of T allele in the case group was higher than that in the control group, and there was significant difference (P<0.05). Conclusion　There is a single base point mutation C>T in the rs13332514 (F353F) locus of ABCA3 exon10. The mutation of this site may be related to the pathogenesis of NRDS in the Han nationality in Western Inner Mongolia. Allele T may increase the susceptibility of NRDS.