Cholelithiasis is being documented with increasing frequency in the paediatric age group. Causes of gallstone formation in infants and neonates seem to differ from those in older children and adolescents. Two infants with pseudohypo-aldosteronism and cholelithiasis are reported. Salt-wasting and dehydration in pseudohypo-aldosteronism are suggested to be the pathogenetic mechanisms leading to gallstone formation possibly beginning in fetal life. The diagnosis of pseudohypo-aldosteronism may be missed, when salt-wasting is transitional. Cholelithiasis may go undetected when asymptomatic. CONCLUSION Pseudohypo-aldosteronism should be considered in infants with cholelithiasis even without obvious salt-wasting signs. Routine ultrasonographic screening for gallstones should be performed in patients with pseudohypo-aldosteronism.