Medullary thyroid carcinoma accounts for 5-10% of all thyroid cancers. It is sporadic in 75% of cases and familial in 25% of cases. Germ-line REarranged during transfection (RET) proto-oncogene mutations are detected in more than 95% of patients with familial medullary carcinoma whereas somatic RET mutations are detected in 40-70% of sporadic medullary carcinomas. Surgery is the only curative treatment and should consist of total thyroidectomy with central and ipsilateral or bilateral lateral lymph node dissection. Surgery provides successful cure in almost 100% of patients when tumor size measures a few millimeters, in almost 90% of patients with a tumor measuring less than 1 cm, and in only 50% of patients with a tumor larger than 1 cm. Alternative forms of treatment involving radiotherapy or chemotherapy provide little benefit. A perspective of recent trials and research into novel treatment of medullary thyroid carcinoma is summarized in the following paper. In this review we examine immunotherapy, radioimmunotherapy, therapy targeting the RET gene or protein, suicide gene therapy, cyclooxygenase inhibitors and radioiodine therapy following sodium iodide symporter gene expression.