The case of a 24-year-old woman with follicular carcinoma arising de novo in a thyroglossal duct is reported. A review of the literature disclosed seven case reports of thyroglossal duct carcinoma with a mixed papillary-follicular pattern. There are three criteria for the diagnosis of follicular carcinoma of a thyroglossal duct: presence of a thyroglossal duct, absence of carcinoma within the thyroid gland and angioinvasion. A plan of treatment, based on the literature survey, should include Sistrunk excision of the tumour if possible, and subtotal thyroidectomy should be performed to eliminate the presence of a primary thyroid carcinoma.