Parathyroid adenomas are a very common endocrine entity, whereas parathyroid carcinomas (PC) are extremely rare. With an estimated incidence of 0.015 per 100,000 population and an estimated prevalence of 0.005% in the United States, parathyroid cancer is one of the rarest of all human malignancies. PC is an extremely rare cause of primary hyperparathyroidism (PHPT). Men and women are equally affected, usually in the fourth or fifth decade of life. The exact etiology has not been identified, but mutations in the CDC73 gene appear to have a critical role in its pathogenesis. The morbidity and mortality of PC is secondary to the hypercalcemia resulting from tumor overproduction of PTH. The primary management objectives are complete removal of the cancer and prevention of recurrences. This review will talk about the epidemiology, etiology, clinical manifestations, and management of PC.