Renal tubular acidosis is a rare form of chronic metabolic acidosis, which is either inherited as an autosomal dominant condition (Types 1, 2, and 3) or acquired. Its effects on pregnancy and vice versa are not known, but chronic acidosis may affect fetal bone growth and development. Chronic maternal acidosis may also lead to fetal distress, which should respond to correction of the maternal acidosis. The patient is a 20-year-old gravida 2, para 1-0-0-1, Hispanic female with distal renal tubular acidosis, diagnosed 1 year prior to this pregnancy after suffering from hypokalemic paralysis. During the pregnancy she required steadily increasing doses of potassium and bicarbonate, to maintain electrolyte balance. She delivered a healthy full-term female infant, weighing 2,892 g, with Apgars of 5 and 9 at 1 and 5 min, respectively, following an induction of labor for oligohydramnios. There was no evidence of intrapartum or neonatal distress, and the infant was discharged home with her mother on the first postpartum day in good health. Established renal tubular acidosis, which was adequately treated with bicarbonate and potassium supplementation during pregnancy, had no apparent ill effects on fetal or neonatal well-being in this case.