Parathyroid carcinoma (PC) is a rare disease and presents clinical challenges in diagnosis, management and adjuvant treatment. Rarely diagnosed preoperatively, PC is often discovered in the treatment of primary hyperparathyroidism. PC is predominantly treated surgically with minimal additional treatments available. Given the rarity of the disease, current and future efforts, as discussed in this article, are focused on increasing preoperative diagnosis, recognizing genetic patterns and pathologic markers and delineating nonsurgical options for patients with advanced disease.