Abstract: Objective: To study KLF7 and CPO gene mutation in a pedigree with autosomal dominant hereditary leukonychia totalis.Methods: All exons of the two genes were amplified using polymerase chain reaction and then were subjected to automatic DNA sequencing to find the mutation.Results: Five mutations were found but the pathopoiesia mutation was not found in all exons and their flanking intronic sequences of the two genes.Conclusion: The diseases of the family with autosomal dominant hereditary leukonychia totalis are not caused by the mutation of exons and their flanking intronic sequences of KLF7 and CPO gene.