Distal renal tubular acidosis (dRTA) is a rare inherited disorder caused by the inability of the kidney to secrete hydrogen ions in the distal tubule. This causes metabolic acidosis with a normal anion gap [1]. In some patients with dRTA, there is sensorineural hearing loss [2]. The mode of inheritance is autosomal recessive. It is most frequently caused by mutations in the ATP6V1B1 or ATP6V0A4 genes. This evidence concerns the gene ATP6V1B1 and distal renal tubular acidosis.