A 43-day old infant with myelomeningocele was evaluated for feeding problems and ill appearance. The presence of metabolic acidosis, hyponatermia and hypercalemia suggested renal tubular acidosis type IV. Later examination showed urinary tract infection together with bilateral vesicoureteral reflux. After treatment of the infection, metabolic acidosis and electrolyte imbalances were resolved and the diagnosis of pseudohypoaldosteronism was confirmed.