This chapter describes the clinical presentation of parathyroid carcinoma (PC) and its variants. The current recommendations for diagnosis and management of patients with PC are described following the most current evidence-based literature. This chapter discusses the clinical factors of preoperative and intraoperative suspicion and the criteria for the unequivocal diagnosis of PC. It also comprehensively describes PC surgical management and the therapeutic goals of resection. A detailed description of the prognostic factors and recommendations for long-term clinical follow-up of patients with PC after their first surgical intervention are included as well. The chapter concludes with a case study along with a series of multiple-choice questions to enhance learning.