This study describes the findings of a retrospective study of 57 medullary thyroid cancer patients observed at the main Sicilian centres for endocrine surgery. Six of these patients were observed locally in order to add a further dimension to the discussion of surgical therapies for medullary thyroid carcinoma. A review of the literature on sporadic forms of medullary thyroid carcinoma and hereditary neoplastic syndromes is followed by presentation of the authors' series. Diagnostic protocols, surgical therapies and pathological observations are discussed. Particular attention is paid to patterns of metastases of medullary thyroid carcinoma and to the differing opinions regarding the extent of lymph-node dissection required and the treatment of recurrent disease. Total extracapsular thyroidectomy with lymph-node dissection is identified as the gold standard of surgical therapies for this form of cancer.