The report describes a patient with coexisting primary hyperparathyroidism due to adenoma of the left superior lobe and the primary empty sella syndrome (ESS). Pathogenetic mechanisms, clinical pictures, associated illnesses and similarity between these diseases are discussed. A complete diagnostic procedure and follow-up is necessary even if the illnesses are oligosymptomatic. In fact this association may be part of multiple endocrine neoplasia (MEN) or other endocrine diseases.