Hyperparathyroidism-jaw tumor syndrome (HPT-JT) is a rare autosomal dominant endocrine neoplastic disorder characterized by increased values of PTH due to neoplasms of parathyroid glands, in 10% to 50% of cases ossifying fibromas of the jaws and in 15% neoplasms of the kidneys and/or the uterus [1, 2]. The gene discussed is PTH; the disease is Hyperparathyroidism-jaw tumor syndrome.