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INTRODUCTION: Parathyroid carcinoma (PC) is a slow-growing and very rare malignancy, representing less than 0.005% of all cancers. The rarity of PC makes it hard to create large-scale published series of patients affected by this condition. MATERIALS AND METHODS: We present four cases of PC treated in our Institution and analyze the most salient aspects of this disease highlighted in recent international literature. DISCUSSION AND COMMENTS: In our experience, in 3/4 cases, the clinical presentation of PC was not different compared to adenoma; therefore, the diagnosis was a post-operative histopathological surprise. We noticed a clear, preoperative laboratory evidence of higher values of PTH and calcemia in patients with PC compared to adenoma. In our experience, a surgical approach consisting in parathyroidectomy associated with ipsilateral loboisthmectomy and central neck lymph node dissection offers the best prognostic chance. Moreover, surgery should be performed only in referral centers where an interdisciplinary management is guaranteed. We are confident in suggesting the surgical approach also in patients with poor clinical status due to high calcemia. If performed by experienced surgeons, the surgical risk of parathyroidectomy is low and we assisted to a progressive restoration of neurological function and an improvement in the patient’s quality of life. Finally, even if our experience is limited, we observed an association between PC and thyroid cancer that deserves a validation through more comprehensive further studies. CONCLUSION: PC remains a complex disease in which a valid surgical approach represents the only curative treatment.