ObjectiveTo investigate diagnosis, gene detection, and treatment principle of medullary thyroid carcinoma.Method The relevant literatures and guidelines about diagnosis and treatment of medullary thyroid carcinoma were summarized and analyzed retrospectively. Resultsmedullary thyroid carcinoma was given priority to surgical treatment. hereditary medullary cancer could be prophylactic thyroidectomy by the RET gene test results. advanced progressive medullary thyroid carcinoma, could be treated by palliative surgery, external radiotherapy, or systemic treatment with the tyrosine kinase inhibitor. ConclusionsPrognosis of medullary thyroid carcinoma is worse, and occurrence of early metastasis is easy. so the first operation should be thoroughgoing. and the operation timing of prophylactic total thyroidectomy for hereditary medullary cancer could be determined by the results of RET gene detection to achieving early cure.