Abstract: Medullary thyroid carcinoma (MTC) is a malignant tumor that originated from the C cells of thyroid. Given its special function, timely radical thyroid operation is the preferred treatment for this tumor. However, most patients are prone to early metastasis, and the sodium/iodide symporter is unexpressed on the C cell surface. Therefore, this tumor cannot be treated with radioactive iodine. In recent years, the clinical application of targeted therapy with tyrosine kinase inhibitors is increasing. Furthermore, radionuclide therapy and other interventional targeted therapies are becoming rapidly developed, which results in an opportunity to manage the aggressive medullary thyroid cancer.