Hereditary thyroid cancer in childhood occurs in the setting of Multiple Endocrine Neoplasia types 2A and 2B, and familial medullary thyroid carcinoma, FMTC. Prophylactic thyroidectomy in childhood has been recommended in patients found to have inherited mutations in the RET protooncogene, to prevent the development of medullary thyroid carcinoma. In this report are presented the clinical and genetic aspects of hereditary thyroid cancer in children, as well as surgical recommendations and medium-term outcome results.