Cystic fibrosis (CF) is an autosomal recessive inherited disease affecting multiple body systems.Pseudo-Bartter syndrome (PBS) is a common manifestation of CF, with such clinical features as hypochloremia, hyponatremia, hypokalemia and metabolic alkalosis.However, PBS patients do not have renal tubulopathy.Children with CF are prone to develop electrolyte abnormalities due to fluid and electrolyte loss.In this article, the pathogenesis, clinical manifestations, diagnosis, and treatment of CF associated PBS were reviewed in order to enhance clinical understan-ding of this disease.