Pseudohypoaldosteronism type 1 (PHA1) is characterized by salt loss which is due to peripheral resistance to aldosterone. Common clinical manifestations of PHA1 include hyponatremia, hyperkalemia, metabolic acidosis and elevated plasma aldosterone levels. The symptoms of PHA1 are easily confused with the symptoms of congenital adrenal hyperplasia associated with 21-hydroxylase deficiency or 3-beta-hydroxysteroid dehydrogenase deficiency; and the symptoms of hypoaldosteronism due to aldosterone deficiency. According to the clinical manifestations and Mendelian inheritance patterns, PHA1 can be classified as either renal PHA1 (autosomal dominant) or the more severe systemic PHAl (autosomal recessive). Herein, we presented two children with PHAl. Two children presented with vomiting, diarrhea, restlessness and dehydratation. After eliminating salt-losing congenital adrenal hyperplasia, PHA was diagnosed with high plasma renin activity and aldosterone.