In this study, compound heterozygous variants (c.1974‐1G>C and c.7787G>A) in the DNAH11 gene were identified in the PCD patient via whole‐exome sequencing and Sanger sequencing, and the heterozygous DNAH11 c.1974‐1G>C and c.7787G>A (p.(Arg2596Gln)) variants were detected in his unaffected daughter and son, respectively. Here, DNAH11 is linked to primary ciliary dyskinesia.