ZNF22 (zinc finger protein 22), also known as KROX-26, is a DNA-binding transcription factor located at chromosome 10.2 1. The protein binds DNA through the consensus sequence 5'-CAATG-3' and functions as a transcriptional regulator 2. During human fetal development, ZNF22 is expressed in the epithelial component of developing tooth organs during early bud and cap stages, as well as in osteoblasts of craniofacial bone and the developing tongue, suggesting a role in tooth formation 2. The ZNF22 gene maps to chromosome 10.21, a locus associated with permanent tooth agenesis (He-Zhao deficiency), further supporting its involvement in odontogenesis 2. ZNF22 belongs to a cluster of zinc finger genes in the pericentromeric region of chromosome 10 1, a region linked to multiple endocrine neoplasia syndromes 1. In clinical applications, ZNF22 has been used as a polymorphic genetic marker for linkage analysis in families with medullary thyroid cancer and MEN2 syndromes 34. Recent whole exome sequencing identified mutations in ZNF22 as probably pathogenic in a case of HPV-related sinonasal carcinoma 5, though the functional significance remains unclear.