We determined the prevalence and clinical features of rate-dependent distal renal tubular acidosis (dRTA) in 31 children examined for possible renal tubular acidosis by measuring the urinary-minus-blood partial pressure of carbon dioxide (U-B PCO2) gradient, minimal urinary pH, and fractional excretion of bicarbonate. Of 20 patients with low U-B PCO2 gradients, nine could not lower urinary pH < or = 5.5, indicating classic dRTA, whereas 11 could lower urinary pH < or = 5.5, as described in rate-dependent dRTA. When patients with rate-dependent dRTA and classic (type I) dRTA were compared, there was no difference in the mean U-B PCO2 gradient or in clinical findings, including age, reason for referral, presence of nephrocalcinosis, or depression of linear growth. We conclude that children with rate-dependent dRTA are susceptible to at least some of the same sequelae as children with classic dRTA. Measurement of minimal urinary pH will not detect this subtle form of dRTA. Determination of the U-B PCO2 gradient should be considered a routine part of evaluation for suspected renal tubular acidosis in a child.