Papillary carcinoma arising in a thyroglossal duct cyst is a rare finding. Less than 100 cases have been reported in the English literature. In most cases the diagnosis is only established after excision of a clinically benign thyroglossal duct cyst. The aetiology of such tumours is unclear but de novo origin and spread from a primary thyroid gland tumour has been suggested. This has important implications for therapeutic approaches. A further case of thyroglossal duct carcinoma is presented and the management is discussed on the basis of the current rationale for treatment of thyroid cancer.