The purpose of this review article is to summarize the salt depletion and metabolic alkalosis (known as pseudo-Bartter syndrome) in cystic fibrosis patients. Although nearly half century passed from the first description of electrolyte imbalance and metabolic alkalosis as a presenting future of cystic fibrosis patients, it is still uncommon. There is no data why some patients develop pseudo-Bartter syndrome and some are not. Published case series focused on initial symptoms, accompanying findings, biochemical abnormalities, feeding differences, age, occurrence rate, seasonal predilection and mutation analysis. And there is no data about the prognosis of and clinical outcome of the cystic fibrosis patients presented with pseudo-Bartter syndrome. In conclusion, this chapter provides relevant information to clinicians about the pathophysiology, prevalence, risk factors and treatment of pseudo-Bartter syndrome in cystic fibrosis patients.