Previous studies have shown that most dRTA cases are caused by mutations in the SLC4A1, ATP6V1B1, and ATP6V0A4 genes, which encode AE1, transmembrane a4, and catalytic b1 subunits of the apical H+-ATPase, respectively.[4] In this study, we report a rare case of dRTA caused by a homozygous deletion of exons 13 and 14 in the ATP6V0A4 gene. This evidence concerns the gene ATP6V0A4 and distal renal tubular acidosis.