Ketotic hypoglycaemia is the most common cause of hy-poglycaemia in preschoolers. The literature does not describe cases that have demonstrated the association between celiac disease and idiopathic ketotic hypoglycaemia. The article reports the case of a 6-year-old girl who presented with feeding problems for several months and frequent episodes of symptomatic hypoglycaemia, not due to metabolic causes (adrenal insufficiency, GH deficien-cy, and glycogen storage disease). The diagnosis of celiac disease was formalized with the biopsy in the presence of a low positivity for transglutaminase. With the onset of gluten-free diet the child has solved the lack of appetite and has not showed more episodes of symptomatic hypo-glycaemia.