Background: Eosinophilic gastrointestinal disorders (EGIDs) are a group of uncommon inflammatory diseases. In this study, we aimed to evaluate the prevalence of EGIDs, clinical manifestation and their response to treatment in a Children Medical Center in Iran. Method: Between 2011 and 2016, all patients, aged <18 years old, who had pathology confirmed diagnosis of EGIDs and underwent endoscopy in our center, were included in the study. Patient demographics, clinical presentation, laboratory data, radiologic findings, endoscopic findings, histological findings and the results of treatment were retrieved from the participants' medical records. Results: The prevalence of EGIDs in children in the gastrointestinal unit of our hospital during these years was reported as 5 per 1000 children. The most reported clinical symptoms in patients with EoE were nausea (75%), vomiting (69%), and epigastric pain (62.5% each). All of the patients had a hypoallergenic diet. Seventy-five percent of the patients used fluticasone spray. The most frequent symptom in the patients with EoC was rectal bleeding (100%), followed by diarrhea (73%), irritability (23%), and loss of weight gain (18%). All of the patients had a hypoallergenic diet. Twenty-seven percent of the patients used Neocate milk and the use of cetirizine and ketotifen were reported in 18% of patients. EoGE was reported in only two patients (mean age: 10.25 years). Conclusion: During the 5-year period, most cases of EGIDs were related to EoC and EoE, and all cases improved in response to treatment. The hypoallergenic diet was identified as a common effective treatment for the studied patients