The prognosis of congenital hyperinsulinemia depends on the type and severity of the hyperinsulinemia that the child has. The most severe and long-lasting complication is brain damage, and children also exhibit low learning ability, episodes of hypoglycemia, and cerebral palsy. Children who are effectively treated with diazoxide have a good long-term prognosis, but require frequent follow-up visits to the hospital to ensure the safety of the medication and to make adjustments to the drug dose. After subtotal pancreatectomy in children with diffuse disease, there is still a risk of hyperinsulinemia, and children may still require frequent feedings and medical medication to stop hypoglycemia after surgery. Some children require multiple pancreatic surgeries due to recurrence, and others may develop permanent type 1 diabetes due to excessive pancreatectomy. Due to the loss of pancreatic enzymes, some children may have impaired intestinal food absorption, and pancreatic enzyme replacement therapy may be indicated if necessary. Children with focal disease can be cured by successful removal of the diseased part of the pancreas. Children with this type are not at risk for diabetes mellitus or impaired intestinal food absorption. In conclusion, with the elucidation of the pathogenesis of CHI in recent years, rapid progress has been made in its diagnosis and treatment. For children diagnosed with CHI, experimental treatment with diazoxide is recommended first. For CHI in which medical treatment is ineffective, the histological type of the pancreas can be predicted by relevant tests so that the correct surgical modality can be selected for treatment. When clinical application of the [18F]-L-DOPA PET Scan technique is difficult, genetic analysis can be used as a complementary predictive tool for diagnostic staging of severe types of CHI.