Parathyroid cancer is a rare endocrine tumor and an uncommon cause of HPT. Advances have been made to identify a promising molecular diagnostic marker for the disease. The use of accurate preoperative imaging modalities would undoubtedly facilitate its management by making an accurate preoperative diagnosis by assessing its invasiveness, and by searching for nodal or distant metastases. The effectiveness of the application of intraoperative PTH assay in the management of this rare condition remains to be seen. Radical surgical treatment offers the best chance of cure, but for patients who have refractory unresectable disease or metastases, the availability of more effective targeted medical therapy may palliate the debilitating symptoms of hypercalcemia, reduce its metabolic complications, and potentially improve survival.