Diagnosing and treating seizures is part of our daily practice. One of the most easily assessed causes is abnormal blood glucose levels. Our aim is to highlight the importance of the differential diagnosis of seizures and hypoglycemia in children in a case study (1-5). Our 1.5-year-old patient had a history of prolonged epileptiform seizures with impaired consciousness due to infectious diseases associated with severe hypoglycemia and metabolic acidosis. These episodes resolved with bolus glucose administration. During endocrinologic evaluation at our hospital, symptomatic hypoglycemia was induced by a fasting test. Routine laboratory tests, repeated metabolic screening, serem amino acids, metabolite profile, urinary organic acids and hormone tests confirmed the presence of ketotic hypoglycemia. If a blood glucose abnormality is confirmed in the background of a seizure, referral to an endocrinology center after acute treatment is necessary to clarify the exact aetiology and to prevent long-term neurological complications of hypoglycemia.  Kulcsszavak: seizure, hypoglycemia, idiopathic ketotic hypoglycemia, endocrinology