Tertiary hyperparathyroidism is intended as a state of excessive autonomous excretion of parathyroid hormone after longstanding secondary hyperparathyroidism. It is a condition of a severe hyperparathyroidism that persists in the renal recipient despite a successful transplantation. The high levels of parathyroid hormone lead to a worsening of graft function accompanied by systemic symptoms. This article reviews the main aspects related to tertiary hyperparathyroidism from mechanism of pathogenesis to prevention and treatment, suggesting some indications about the best management of patients on a waiting list for kidney transplant.