Parathyroid carcinoma is a rare endocrine malignancy and an uncommon cause of PTH-dependent hypercalcemia. Mutations in CDC73 play a key role in its etiopathogenesis. Its presentation is similar to that of patients with a parathyroid adenoma; however, the presence of palpable neck mass, voice change, hypercalcaemic crisis or severe multiorgan involvement should raise the suspicion of parathyroid carcinoma. The high level of serum calcium is the common cause of morbidity in these patients, which requires urgent medical management. Localisation studies include ultrasound of the neck and 99mTc sestamibi scan. Contrast-enhanced computerised tomography or MRI is recommended in patients with clinical suspicion of local infiltration. Surgery is the mainstay of treatment involving en bloc resection. Histopathological diagnosis is challenging; however, IHC with parafibromin seems to aid in the diagnosis. The role of adjuvant radiation therapy and chemotherapy is limited. Targeted therapy is reserved for patients with locally advanced or metastatic disease.