Cancer of the parathyroid gland is rare and most patients suffer from hyperparathyroidism (HPT). Therefore it is often difficult to distinguish between benign HPT and malignant disorders. Prognosis as well as therapeutic approaches are still limited. Surgical treatment should be considered the therapy of choice for primary and recurrent manifestation of parathyroid carcinoma. The initial operation includes en-bloc resection of the tumor and the ipsilateral thyroid lobe. Surgery may cure some patients, but is often only palliative and aims to control hypercalcemia. In some patients hypercalcemia may be refractory to repeated surgery and medical treatment can provide short- or middle-term effects.