The case of a patient with a large goitre associated with hypercalcitoninaemia and fasting hypoglycaemia is reported. Pentagastrin (PG) test was negative. Repeated measurements of fasting glycaemia, insulin and C peptide established the diagnosis of insulinoma. After localization by endoscopic ultrasonography, a distal pancreatectomy was performed and a small insulinoma was recovered. Glycaemia and calcitonin (CT) became normal. The tumour cells displayed a strong immunoreactivity for insulin and CT. Cytogenetical evaluation of the tumour revealed a translocation t(1;9) (p13;p22).