We report a case of macroamylasemia in an 11-year-old boy. We compare our clinical and paraclinical data with those described in pediatric literature. Macroamylase resulted in a complex of amylase and immuglobulin. Its fortuitous detection did not reveal, up to now, any associated pathology, in particular any autoimmune disorders or celiac disease. Identification of this biochemical abnormality is essential in order to avoid invasive investigations and/or unnecessary therapies.