Sporadic persistent hyperinsulinemic hypoglycaemia in a newborn is a rare disease which requires early surgical treatment if conservative therapy fails. We report on a newborn female in whom PHHI was diagnosed at the second day of life. She presented with severe hypoglycemia and high levels of plasma insulin. Conservative treatment failed and a near-total pancreatectomy was performed on the 24th day of life. The postoperative outcome and the follow-up studies showed the patient to be euglycaemic. If hypoglycaemia is resistant to conservative therapy, surgical treatment is recommended. A loss of pancreatic exocrine function is not necessarily to be expected.

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