Abstract: Anaplastic thyroid cancer (ATC) is a rare thyroid tumor, accounting for approximately 1%-2% of all thyroid malignancies. It is the most aggressive type of thyroid cancer, resulting in significant morbidity and mortality. Older age, male, bilateral tumors, local invasion, and/or distant metastasis are adverse prognostic factors for most patients with ATC. The best treatment strategy for ATC remains unknown. Currently, comprehensive treatments such as surgery, radiotherapy, chemotherapy, targeted therapy, and immunotherapy are mainly used; however, the current treatment status remains unclear. By reviewing relevant studies on ATC in China and other countries, this review summarizes the current diagnosis, treatment plans, recent clinical trial results, and future research directions to provide references for ATC diagnosis and treatment decision making.