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Congenital hyperinsulinism (CHI) is a cause of severe hypoglycemia in the neonatal and infancy period. Histologically, there are two subtypes with diffuse and focal disease. The preoperative differentiation of these two forms is very important because the surgical management is radically different. The focal form of the disease can be cured if the focal lesion can be localized accurately and completely resected with surgery.We report the case of a child who underwent three pancreatectomies with a choledochoduodenostomy and a cholecystectomy but continued to have severe hyperinsulinemic hypoglycemia.Radiological investigations including imaging with 18fluoro-l-Dopa positron emission tomography scan showed a clear focus of increased 18F-fluoro-l-Dopa uptake in the vicinity of the former head of the pancreas. On the magnetic resonance imaging scan, this focal uptake appeared to localize adjacent or next to duodenum (in the wall or cavity of the duodenum).This unique case highlights the importance of correctly localizing and completely resecting the focal lesion in patients with CHI. 18Fluoro-l-Dopa positron emission tomography scan can identify ectopic focal lesions in patients with CHI.