Abstract: Advanced progressive medullary thyroid carcinoma (MTC) is usually characterized by rapid progression, poor prognosis, and limited therapeutic options, so there is an urgent need to develop a novel and effective therapy. In recent years, the integrated precision diagnosis and treatment of nuclear medicine guided by molecular imaging, such as the precise diagnosis and therapy of neuroendocrine neoplasms (NEN) targeting somatostain receptors (SSTR), has been developed rapidly and gained great success. MTC classified as a subtype of NEN, which can specifically overexpress SSTR, fibroblast activation protein, cholecystokinin-2 receptor, and prostate-specific membrane antigen, making them the ideal targets for the precise diagnosis and treatment of MTC. In this review, the authors will review the current situation and advances of the theranostics of MTC guided by molecular imaging.