Background: Hyperinsulinism is the most common cause of persistent or recurrent neonatal hypoglycemia that may result in neurological deficits. The treatment goal in these patients is prevention of hypoglycemia to decrease mortality and morbidity. This study was done to determine the clinical course and outcome in children with congenital hyperinsulinism (CHI) referring to Mofid Children’s Hospital from 2011 to 2017. Methods: This study was done on 22 children with CHI referring to Mofid Children’s Hospital from 2011 to 2017. The demographic, perinatal, clinical, laboratory, imaging, pharmacological, treatment and follow up data of these children were collected and analyzed. Results: Among 22 children with CHI, the mortality rate was higher among those who received hydrocortisone versus those who did not receive hydrocortisone (46% versus 40%). Conclusion: According to the results of this study, hydrocortisone had a negative impact on the outcomes of these children, which is important in the management of hypoglycemia. The clinical course and outcome of children with CHI was better with medical compared to surgical treatment.