Persistent hyperinsulinemic hypoglycemia in neonatal period is characterized by insulin hypersecretion. The major feature is severe hypoglycemia, generally unresponsive to routine medical treatment. Subtotal or total pancreatectomy is performed in unresponsive cases. In this case report, we present a newborn with persistent hypoglycemia unresponsive to medical treatment with dysrhythmic left ventricular hypertrophy. The insulin/C-peptide ratio was 58 as a confirmation of diagnosis. Since hypoglycemia persisted after the initial medical treatment, a subtotal pancreatectomy was performed followed by near-total pancreatectomy. A histologic examination revealed diffuse insulin islets. At the 70th post-natal day, death occurred due to heart failure and ventricular dysrhythmia. To our knowledge, severe dysrhythmia and left ventricular hypertrophy in persistent hyperinsulinemic hypoglycemia (PPH) is identified in the patient.