Parathyroid carcinoma is a rare endocrine tumour accounting for only about one of every hundred cases of primary hyperparathyroidism. The aetiology of this cancer remains obscure but the recent studies have identified that some gene mutations may be involved in its pathogenesis. Most patients with parathyroid carcinoma suffer from symptoms related to marked hypercalcemia; the incidence of associated renal, bone, gastrointestinal, neuromuscular and psychological symptoms is much more frequent than in those with benign parathyroid adenomas. The course of patients with parathyroid carcinoma is variable. However, in more than 50% cases, patients experience persistent or recurrent disease due to regional or distant disease. The treatment of parathyroid malignancy is predominantly surgical, comprising an initial en bloc resection of the tumour and adjacent neck structures. Several studies have suggested the usefulness of pharmacotherapy in the palliative treatment of the debilitating symptoms of hypercalcemia. The aim of this paper is to summarise the present state of knowledge on the aetiology, clinical presentation, diagnosis and treatment of parathyroid carcinoma.