Distal renal tubular acidosis is usually primary (genetic) in children and is associated with mutations in five genes: ATP6V0A4, ATP6V1B1, SLC4A1, FOXI1, and WDR72. It may also develop secondary to systemic pathologies, drug intake or exposure to toxic substances [1, 2]. Here, ATP6V0A4 is linked to distal renal tubular acidosis.