Abstract: Medullary thyroid carcinoma (MTC) is a type of neuroendocrine tumor that originates from calcitonin-producing parafollicular C cells of the thyroid. It is a moderate malignant thyroid carcinoma with a high recurrence rate and an early metastasis rate. The common diagnostic methods include detection of calcitonin, and tumor markers (such as carcinoembryonic antigen), ultrasound, fine needle aspiration cytology(FNAC), which have low specificity. And FNAC has high technical requirements. Radical surgery is currently the first choice of treatment of MTC. However, invasive and metastatic lesions are difficult to completely remove. Procalcitonin and novel molecular targeting probes provide new ideas for the early diagnosis and effective treatment of MTC. This review comprehensively discussed the recent advances in the diagnosis and treatment of this disease.