Medullary thyroid carcinoma arises from the C cells, which produce a characteristic hormone, calcitonin. At present, surgery is the main treatment modality. Medullary thyroid carcinoma is usually treated with total thyroidectomy and with removal of nodes in the central portion of the neck and upper mediastinum. Cervical nodes dissection may be required for cancers affecting lateral neck nodes. Substitutive therapy with levo-thyroxine is indicated after surgery. External beam radiation therapy is not effective against advanced medullary thyroid carcinoma, while chemotherapy has a marginal activity. Biological therapy induces its anti-tumour activity through the inhibition of tumour cell growth without cytolysis and stimulating the antitumour immune response, in the absence of relevant side effects. On these bases, it can be suggested that chemo-refractory tumours could be still responsive to biological agents. In the last years somatostatin analogues and interferon have been used in the therapy of advanced and symptomatic medullary thyroid carcinoma, demonstrating an efficacious effect on neuroendocrine symptoms and on the production of calcitonin and an improving in quality of life. Even if there are no consistent data on the effects of biological agents on the reduction of tumour mass, the combined use of chemotherapy and biological therapy needs to be experimented in medullary thyroid carcinoma.