Background: Anaplastic thyroid cancer (ATC) is an orphan disease with an extremely poor prognosis. Radical surgery is possible only for stage IVA anaplastic thyroid cancer, and no effective treatments are available for stage IVB or IVC anaplastic thyroid cancer. The response rate of the disease to cytotoxic chemotherapy has been previously reported as unsatisfactory. Objective: In 2015, lenvatinib was approved and reimbursed for anaplastic thyroid cancer in Japan, including radioactive iodine-refractory differentiated thyroid cancer. Because of its rarity, only a few reports exist regarding the collective treatment outcomes of lenvatinib for anaplastic thyroid cancer. Methods: A comprehensive literature review was finalized in September 2018. PubMed® was used for research. Electronic search results were supplemented via hand searching of selected papers, expert consensus meeting notes, and reference lists from selected articles. Results:We reviewed the progress of the chemotherapy for ATC before the tyrosin kinase inhibitor (TKI) treatment and collected the reports regarding the treatment outcomes of lenvatinib for ATC. Conclusions: In this study, we reviewed the outcomes of chemotherapy for anaplastic thyroid cancer and assessed the role of tyrosine kinase inhibitor therapy in future treatment.