Undifferentiated or anaplastic carcinoma is an uncommon histologic type of thyroid cancer. It is one of the most aggressive malignancies associated with a poor prognosis. Most patients are elderly presenting as locally advanced disease with nodal and distant metastases. Complete surgical resection is frequently not possible and there is no effective systemic therapy. Aggressive multimodal therapy including surgery, radiation, and chemotherapy is recommended for management. However, because of the rarity of the disease, its aggressiveness and the lack of prospective treatment protocols, nearly all evidence in the literature comes from retrospective case series or cohort studies for selected patients' subgroups treated over a relatively long study period. Evidence of therapeutic benefit of one treatment option over another is lacking. Prognosis remains dismal with a median survival of 2-12 months Surgery remains an important component of the multimodal therapy and is commonly adopted as primary treatment. Although radical resection should be discouraged, resection without inducing significant morbidity can be considered for good risk patients with resectable tumors. It provides an effective form of palliation with potential cure when combined with postoperative radiotherapy and/or chemotherapy. Patients selected for surgical resection was frequently identified to have improved survival. A regime of preoperative hyperfractionated radiotherapy and doxorubicin followed by surgery when feasible has been documented to achieve local control and avoid tracheostomy for ATC patients. Because of the ineffectiveness of all conventional treatment modalities, novel molecular targeted therapies are being developed to tackling this uniformly fatal disease with promising results.