Background: Congenital hyperinsulinism (CHI) is the most common cause of persistent hypoglycemia in neonates and children. Prompt diagnosis and treatment is important to avoid long-term neurological damage. In diazoxide-unresponsive cases, somatostatin receptor analogs are used in second line. Pasireotide, which has a higher affinity for the somatostatin receptor 5 than first-generation somatostatin analogs, was trialed in a pediatric patient with hyperinsulinism. Case description: The patient presented at the age of 8 years with recurrent episodes of nonfasting hypoglycemia and neuroglycopenic symptoms. A diagnosis of autonomous insulin secretion was biochemically confirmed by elevated levels of insulin (22.9 µUI/mL > 3 µUI/mL) and C-peptide (3.43 ng/mL > 0.6 ng/mL) during hypoglycemia (glucose 2.03 mmol/L). Glucose infusion rate to maintain euglycemia was 7 (mg/kg/min). The patient was initially treated by frequent feeding with carbohydrate-enriched formula and diazoxide 10 mg/kg/day. At the age of 12 years treatment with subcutaneous injections of octreotide was added because of recurrent hypoglycemia. Six months later and with parental consent, long acting pasireotide 40 mg every 28 days was initiated. Common side effects associated with pasireotide were carefully monitored, but did not occur. The patient was normoglycemic with a good growth rate, normal weight gain, and excellent neurodevelopment. Conclusion: This case study adds to findings reported previously suggesting that pasireotide may be a valuable option in second-line treatment of hyperinsulinism.