Abstract Background Parathyroid carcinoma is a rare malignancy affecting 0·5–5 per cent of all patients with primary hyperparathyroidism. This article reviews the literature on the pathogenesis, pathology, clinical features, diagnosis and management of parathyroid carcinoma. Methods A Medline search was performed and all relevant English language articles published between 1970 and 2005 were retrieved. The search words included ‘parathyroid carcinoma’, ‘pathology’, ‘genetics’, ‘management’ and ‘radiotherapy’. Secondary references were obtained from key articles. Results and conclusion The exact aetiology of parathyroid carcinoma remains obscure. Recently, the HRPT2 gene has been implicated in its pathogenesis and may prove to be a genetic target in future. Surgical resection is the accepted ‘gold standard’. There is now a growing consensus on the use of adjuvant radiotherapy as it has been shown to provide a survival benefit.