Individuals with hereditary distal renal tubular acidosis (dRTA) typically present in infancy with poor weight gain and growth deficiency, although later presentations can occur, especially in individuals with autosomal dominant SLC4A1-related dRTA. Initial clinical manifestations can also include emesis, polyuria, polydipsia, constipation, diarrhea, decreased appetite, and episodes of dehydration. Electrolyte manifestations include hyperchloremic non-anion gap metabolic acidosis and hypokalemia. Renal complications of hereditary dRTA include nephrocalcinosis, nephrolithiasis, medullary cysts, and impaired kidney function. Additional manifestations include bone demineralization (rickets, osteomalacia), sensorineural hearing loss (in ATP6V0A4-, ATP6V1B1-, and FOXI1-related dRTA), hereditary hemolytic anemia (in some individuals with SLC4A1-related dRTA), and amelogenesis imperfecta (in WDR72-related dRTA).