Introduction: Pseudo-Bartter syndrome (PBS) is characterized by hyponatremic, hypochloremic metabolic alkalosis that mimics Bartter syndrome. We reviewed the clinical and laboratory features and treatment approaches of 52 children with Cystic Fibrosis (CF) and PBS who evaluated by our institution from 2007 to 2016. Forty-five of these patients continued to follow-up in our clinic. Results: A total of 52 patients with PBS were identified. The median age of the first attack was 2 (1-39) months. Twenty-six (50%) of 52 patients were diagnosed as PBS at the time of the first diagnosis of CF and 26 (50%) patients developed PBS during their follow-up. The median follow-up period was 34.5 months. The episodes of PBS occurred during summer in 17 (35.4%) patients and during winter in 17 (35.4%) patients. Mean pH and bicarbonate values at first attack were 7.54±0.06 and 37.21±7.19 mmol/L on the blood gas analysis. Mean sodium, potassium, chloride, urea and creatinine levels were 128±5.3 meq/L, 3.33±0.6 meq/L, 79.28±12.6 meq/L, 14.83±10.96 mg/dL, and 0.33±0.18 mg/dL. Twenty-three (51.1%) patients had recurrent episodes of PBS. The total number of attack was 81 in 45 patients who were continued to be followed-up in our institution. As an initial symptom, exacerbation of cough was significantly higher in group with recurrent attacks. Other clinical features like failure to thrive, dehydration status and liver disease did not differ significantly between patients who had recurrent attacks and others. Conclusion: Sometimes PBS may be the initial presentation of the disease. According to our results, almost half of the patients experience recurrent attacks. Therefore close follow-up of the CF patients with PBS attacks is vital.