AIM: Parathyroid carcinoma (PC) represents a rare cause of primary hyperparathyroidism (PHPT). In this paper, among patients who underwent surgery for PHPT, we compared those with benign parathyroid disease with those affected by PC in terms of demographic and preoperative biochemical features. Moreover, we singularly described all 10 cases of PC treated at our Institution (including a case that occurred in a patient with tertiary hyperparathyroidism) and a brief review of the literature. MATERIAL AND METHODS: Patients undergoing surgery for PHPT in our Unit between 2003 and 2018 were retrospectively analysed. They were divided into two groups: Group A (benign parathyroid disease), Group B (PC). The case of PC that occurred in the patient with tertiary hyperparathyroidism was not included into the two groups. RESULTS: Three hundred and eight patients were included: 299 in Group A and 9 in Group B. The mean preoperative serum PTH value and mean preoperative serum calcium level were significantly higher in Group B than in Group A (P = 0.018, P = 0.027; respectively). Including the case of PC that occurred in the patient with tertiary hyperparathyroidism, 10 patients with PC were treated at our Institution. Among these, 3 underwent a re-exploration. Disease recurrence occurred in 1 (10%) patient, who developed a local recurrence and distant metastases. CONCLUSIONS: In the presence of PHPT characterized by particularly high preoperative levels of serum PTH and calcium this malignancy should be suspected. On the basis of our experience, we believe that extensive surgery is not always necessary.