The presentation of parathyroid carcinoma in patients with chronic renal failure is rare, although with improvements in life expectancy associated with this condition there have now been 12 reported cases, including the first case we report here. It has been proposed that in these cases there has been a malignant transformation of benign parathyroid hyperplastic tissue. We also report the first case of parathyroid carcinoma associated with coeliac disease and suggest that the same mechanism may be responsible. We review the presentation, diagnosis, treatment and natural history of the disease.