Most cases of primary hyperparathyroidism are due to either a parathyr
oid adenoma or to parathyroid hyperplasia. Parathyroid carcinoma is a
very rare cause of hyperparathyroidism. Although the diagnosis of para
thyroid carcinoma is usually established based on pathological criteri
a of vascular and capsular invasion, some clinical and biochemical fea
tures differentiate it from benign forms of hyperparathyroidism, We re
port the case of a middle-aged woman with a long standing history of n
ephrolithiasis, who presented with a palpable neck mass, weight loss,
severe hypercalcemia and hypophosphatemia, as well as very high serum
levels of intact parathyroid hormone. Surgical neck exploration reveal
ed a large tumor that invaded trachea, esophagus, reccurrent laryngeal
nerve, right apical pleura and right carotid artery, Pathological exa
mination confirmed the invasive nature of the tumor. Along with the ca
se report, we review the literature and discuss the diagnostic and the
rapeutic options of this rare condition.