Introduction: Congenital hyperinsulinism (CHI) is the most common cause of persistent hypoglycemia in neonates and infants, with an estimated incidence of 1:30,000-50,000 live births. It results from inappropriate insulin secretion, leading to suppressed ketogenesis and lipolysis, and poses a significant risk for irreversible neurological damage. Early diagnosis and treatment are essential; however, limited access to genetic and imagin studies in resource-constrained settings hampers appropriate management.Case Description: We report a term male neonate (38 weeks, birth weight 4,345g) born to a mother with polyhydramnios and urinary tract infection. Shortly after birth, he presented with symptomatic hypoglycemia (plasma glucose 1mg/dL), lethargy, tremors, and decreased reflexes. Despite high glucose infusion rates and diazoxide therapy up to 20 mg/kg/day, hypoglycemia persisted.Echocardiography revealed multiple cardiac anomalies (PFO, ASD, VSD,PDA), and cranial ultrasound showed bilateral thalamic vasculopathy. Persistent seizures and respiratory failure required mechanical ventilation. Due to limited resources, 18F-DOPA PET imaging and genetic testing were not performed. The patient developed multi-organ complications and passed away following cardiorespiratory arrest on day 46. (Table 1)Discussion: Refractory CHI is frequently associated with KATP-channel mutations, often requiring surgical intervention. Lack of access to genetic testing and advanced imaging delays appropriate treatment decisions, including ocreotide use or pancreatectomy. This case illustrates the high morbidity and mortality of untreated or refractory CHI, emphasizing the need for early diagnosis and multidisciplinary care.Conclusion: CHI requires prompt recognition, metabolic and genetic evaluation, and specialized management to prevent severe neurological and systemic complications.
